MedWorm.com provides a medical RSS filtering service. Over 6000 RSS medical sources are combined and output via different filters. This feed contains the latest items from the 'Haematologica' source. Wed, 08 Feb 2012 17:42:01 +0100 http://www.medworm.com/index.php?rid=5627362&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271886%26dopt%3DAbstract Conclusions. This detailed analysis clarifies the impact of genetic modifiers on clinical severity of the disease measured by time to first transfusion by determining their relative contributions in a homogeneous cohort of beta0-thalassemia patients. It may also support clinical decisions regarding the beginning of transfusion therapy in patients with beta-thalassemia. PMID: 22271886 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627362 Sun, 22 Jan 2012 05:00:00 +0100 5627362 http://www.medworm.com/index.php?rid=5627361&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271887%26dopt%3DAbstract In this report we we compared the ability to capture prognostic information on comorbidity in MDS by general comorbidity measures (ACE-27 scale) versus disease-specific comorbidity measures (MDS-CI) in 840 consecutive patients diagnosed at our institution between 1992 and 2007. For all comorbid conditions considered in the analysis, clinical definition and grading of severity by MDS-CI resulted more fit for capturing the prognostic information of these comorbid conditions in MDS patients compared to ACE-27. PMID: 22271887 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627361 Sun, 22 Jan 2012 05:00:00 +0100 5627361 http://www.medworm.com/index.php?rid=5627360&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271888%26dopt%3DAbstract Conclusions. PaCSs are a prominent feature of neutrophils from Shwachman-Diamond syndrome patients. They may help us to understand the mechanism of granulocyte dysfunction and the neoplastic risk of the disease. PMID: 22271888 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627360 Sun, 22 Jan 2012 05:00:00 +0100 5627360 http://www.medworm.com/index.php?rid=5627359&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271889%26dopt%3DAbstract Conclusions. We did not confirm that non-Human Leukocyte Antigen polymorphisms were associated with outcomes in myeloablative unrelated donor hematopoietic cell transplantation for Chronic Myeloid Leukemia, possibly due to the strong association between clinical variables and outcome that masked more subtle genetic effects. PMID: 22271889 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627359 Sun, 22 Jan 2012 05:00:00 +0100 5627359 http://www.medworm.com/index.php?rid=5627358&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271890%26dopt%3DAbstract Conclusions: This study demonstrates that NK-92 and KHYG-1 are capable of killing clonogenic and bulk multiple myeloma cells. In addition, multiple myeloma tumor burden in a xenograft mouse model was reduced by intravenous NK-92 cell therapy. Since multiple myeloma colony frequency correlates with survival, our observations have important clinical implications and suggest that clinical studies of NK cell lines to treat MM are warranted. PMID: 22271890 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627358 Sun, 22 Jan 2012 05:00:00 +0100 5627358 http://www.medworm.com/index.php?rid=5627357&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271891%26dopt%3DAbstract Conclusions. Altogether, these results suggest that FoxM1 is over-expressed in a majority of diffuse large B-cell lymphoma samples. These data also indicates that targeting FoxM1 signaling can serve as a potential therapeutic modality in the management of diffuse large B-cell lymphoma. PMID: 22271891 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627357 Sun, 22 Jan 2012 05:00:00 +0100 5627357 http://www.medworm.com/index.php?rid=5627356&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271892%26dopt%3DAbstract Conclusions. In BALB/c mice, gelsolin deficiency alters the erythrocyte actin polymerization/depolymerization equilibrium, causing impaired terminal maturation. We suggest a non-redundant role for gelsolin in terminal erythroid differentiation, possibly contributing to the Gsn-/- mice lethality observed in mid gestation. PMID: 22271892 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627356 Sun, 22 Jan 2012 05:00:00 +0100 5627356 http://www.medworm.com/index.php?rid=5627355&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271893%26dopt%3DAbstract CONCLUSIONIn patients with relapsed/refractory HL responding to prior salvage therapy, PET response at time of ASCT favorably influences outcome and allows to identify patients requiring single or tandem ASCT. PMID: 22271893 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627355 Sun, 22 Jan 2012 05:00:00 +0100 5627355 http://www.medworm.com/index.php?rid=5627354&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271894%26dopt%3DAbstract Conclusions. We show that human CD34+ cells expressing PDGF receptor fusion oncogenes proliferate autonomously and differentiate towards the eosinophil lineage in a process that requires NF-κB. These results suggest new treatment possibilities for imatinib-resistant myeloid neoplasms associated with PDGF receptor mutations. PMID: 22271894 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627354 Sun, 22 Jan 2012 05:00:00 +0100 5627354 http://www.medworm.com/index.php?rid=5627353&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271895%26dopt%3DAbstract ConclusionsOur results implicate microRNA126 in the regulation of hematopoietic stem/progenitor cells trafficking between the bone marrow and peripheral sites, clarify the role of vascular cell adhesion molecule 1in G-CSF-mediated mobilization, and have important implications for improved approaches to selective mobilization of hematopoietic stem/progenitor cells. PMID: 22271895 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627353 Sun, 22 Jan 2012 05:00:00 +0100 5627353 http://www.medworm.com/index.php?rid=5627352&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271896%26dopt%3DAbstract Conclusions:Fc gamma receptor 3A polymorphism status may be predictive of survival in follicular lymphoma patients given treatments containing an anti-CD20 antibody but not treatment with chemotherapy alone. Thus, Fc gamma receptor 3A polymorphisms may be important to consider in designing new follicular lymphoma trials and new anti-CD20 monoclonal antibodies.(clinicaltrials.gov identifier: NCT00933127). PMID: 22271896 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627352 Sun, 22 Jan 2012 05:00:00 +0100 5627352 http://www.medworm.com/index.php?rid=5627351&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271897%26dopt%3DAbstract Conclusions. These results suggest that nelfinavir used at pharmacological dosage, alone or in combination, may be useful in the treatment of myeloma. Our data provide a preclinical basis for clinical trials using nelfinavir in patients with myeloma. PMID: 22271897 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627351 Sun, 22 Jan 2012 05:00:00 +0100 5627351 http://www.medworm.com/index.php?rid=5627350&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271898%26dopt%3DAbstract Conclusions: The unfavorable trend in outcome for adolescent and young adult with chronic myeloid leukemia is unexpected. Additional research in this population is required to better define outcomes, understand the cause of this difference, and to help make better treatment recommendations. PMID: 22271898 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627350 Sun, 22 Jan 2012 05:00:00 +0100 5627350 http://www.medworm.com/index.php?rid=5627349&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271899%26dopt%3DAbstract Authors: Roques M, Durand C, Gautier R, Canto PY, Petit-Cocault L, Yvernogeau L, Dunon D, Souyri M, Jaffredo T Abstract CD105 is an auxiliary receptor for the Transforming Growth Factor Beta superfamily, highly expressed on proliferating endothelial cells and adult hematopoietic stem cells. Because CD105 mRNA expression was reported in the developing aortic region, we further characterized its expression profile in the aorta and examined the hematopoietic potential of CD105+ cells.Aortic endothelial cells, intra-aortic hematopoietic cells clusters and the purified cell fraction enriched in progenitor/hematopoietic stem cell activity expressed CD105. Aortic hematopoietic short-term clonogenic progenitors were highly enriched in the CD105intermediate population whereas more immature ... Haematologica journals http://www.medworm.com/rss/comments.php?id=5627349 Sun, 22 Jan 2012 05:00:00 +0100 5627349 http://www.medworm.com/index.php?rid=5627348&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271900%26dopt%3DAbstract Authors: Stephens D, Ruppert AS, Blum K, Jones J, Flynn J, Johnson A, Ji J, Phelps M, Grever M, Byrd J Abstract Older chronic lymphocytic leukemia patients have poor outcomes with standard treatments and are underrepresented in clinical trials. We retrospectively reviewed outcomes of refractory chronic lymphocytic leukemia patients in two age categories [≥70, <70 years] treated with single-agent flavopiridol, a drug active in genomically high-risk patients, during two trials. No significant difference between older and younger patients was observed in response rates (43% versus 47%) or progression-free survival (median=8.7 versus 9.9 months, p>0.80). Although overall survival was worse in older patients (median=2.1 versus 2.4 years, p=0.02), when adjusted for other factors,... Haematologica journals http://www.medworm.com/rss/comments.php?id=5627348 Sun, 22 Jan 2012 05:00:00 +0100 5627348 http://www.medworm.com/index.php?rid=5627347&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271901%26dopt%3DAbstract Conclusions. Selective addition of day 15 marrow response to conventional stratification criteria applied on ALL-BFM 95, currently in use as regular chemotherapy protocol for childhood acute lymphoblastic leukemia in several countries, may significantly improve risk-adapted treatment delivery. Even though cutting-edge trial risk stratification is meanwhile dominated by minimal residual disease evaluation, an improved conventional risk assessment, as presented here, could be of great importance to countries lacking the technical and/or financial resources associated with the application of minimal residual disease analysis. PMID: 22271901 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627347 Sun, 22 Jan 2012 05:00:00 +0100 5627347 http://www.medworm.com/index.php?rid=5627346&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271902%26dopt%3DAbstract Authors: Bödör C, Renneville A, Smith M, Charazac A, Iqbal S, Etancelin P, Cavenagh J, Barnett MJ, Kramarzova K, Krishnan B, Matolcsy A, Preudhomme C, Fitzgibbon J, Owen C Abstract While most myelodysplastic syndrome/acute myeloid leukemia cases are sporadic, rare familial cases occur and provide insight into leukemogenesis. The most clearly defined familial cases result from inherited mutations in RUNX1 or CEBPA. Recently, novel germline mutations in GATA2 were reported. We thus investigated individuals from families with ≥ 1 first-degree relative with myelodysplastic syndrome/acute myeloid leukemia with wildtype RUNX1 and CEBPA, for GATA2 mutations. Screening for other recurrent mutations was also performed. A GATA2 p.Thr354Met mutation was observed in a pedigree in which 2 f... Haematologica journals http://www.medworm.com/rss/comments.php?id=5627346 Sun, 22 Jan 2012 05:00:00 +0100 5627346 http://www.medworm.com/index.php?rid=5627345&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271903%26dopt%3DAbstract Conclusions. Our results show the presence of altered phenotypes by flow cytometry on platelets from around half of myelodysplastic syndromes patients; such findings may contribute to refine the diagnostic and prognostic assessment of this group of disorders if confirmed in larger series of patients. PMID: 22271903 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627345 Sun, 22 Jan 2012 05:00:00 +0100 5627345 http://www.medworm.com/index.php?rid=5627344&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271904%26dopt%3DAbstract Conclusions. Whereas new electrocardiographic abnormalities were recorded in twenty percent of all patients and some of them developed severe or even life-threatening coronary artery disease, QT prolongation, changes in left ventricular ejection fraction, and clinical cardiac adverse events were uncommon in patients treated with nilotinib. PMID: 22271904 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627344 Sun, 22 Jan 2012 05:00:00 +0100 5627344 http://www.medworm.com/index.php?rid=5627343&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22271905%26dopt%3DAbstract Conclusions. Three years of deferasirox treatment along with a clinically manageable safety profile significantly reduced cardiac iron overload versus baseline and normalized T2* in 68.1% (32/47) of patients with T2* 10 to <20ms. PMID: 22271905 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5627343 Sun, 22 Jan 2012 05:00:00 +0100 5627343 http://www.medworm.com/index.php?rid=5568916&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22210325%26dopt%3DAbstract Authors: Cools J PMID: 22210325 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5568916 Sun, 01 Jan 2012 05:00:00 +0100 5568916 http://www.medworm.com/index.php?rid=5568915&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22210326%26dopt%3DAbstract Authors: De Keersmaecker K PMID: 22210326 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5568915 Sun, 01 Jan 2012 05:00:00 +0100 5568915 http://www.medworm.com/index.php?rid=5568914&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22210327%26dopt%3DAbstract Authors: Tiacci E, Grossmann V, Martelli MP, Kohlmann A, Haferlach T, Falini B PMID: 22210327 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5568914 Sun, 01 Jan 2012 05:00:00 +0100 5568914 http://www.medworm.com/index.php?rid=5568913&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22210328%26dopt%3DAbstract Authors: Rossi D, Gaidano G PMID: 22210328 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5568913 Sun, 01 Jan 2012 05:00:00 +0100 5568913 http://www.medworm.com/index.php?rid=5568909&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22210329%26dopt%3DAbstract Authors: Davidoff AJ, Weiss Smith S, Baer MR, Ke X, Bierenbaum JM, Hendrick F, McNally DL, Gore SD Abstract Patient and physician characteristics associated with use of erythropoiesis-stimulating agents in myelodysplastic syndrome patients have not yet been described. Myelodysplastic syndrome patients diagnosed from 2001 to 2005 were identified from the Surveillance Epidemiology and End Results-Medicare database. Multivariate regressions examined the association between patient and physician characteristics and the probability of receiving any erythropoiesis-stimulating agents, and of receiving therapeutic-length (≥8 week) treatment episodes. Among the 6,588 myelodysplastic syndrome patients studied, 65% received erythropoiesis-stimulating agents. Use of erythropoiesis-stimulatin... Haematologica journals http://www.medworm.com/rss/comments.php?id=5568909 Sun, 01 Jan 2012 05:00:00 +0100 5568909 http://www.medworm.com/index.php?rid=5568903&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22210330%26dopt%3DAbstract Authors: Castagnola E, Haupt R PMID: 22210330 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5568903 Sun, 01 Jan 2012 05:00:00 +0100 5568903 http://www.medworm.com/index.php?rid=5552952&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207681%26dopt%3DAbstract Conclusions: The data support the interpretation and therapy-related precursor B-acute lymphoblastic leukemia does occur. In particular, cases associated with t(4;11)(q21;q23) or hypodiploidy with -5, -7, -17 are likely to be therapy-related and have a poor prognosis. The inferior outcome of these patients may be attributable to the high-risk cytogenetic abnormalities that are found in this group of patients. PMID: 22207681 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5552952 Thu, 29 Dec 2011 05:00:00 +0100 5552952 http://www.medworm.com/index.php?rid=5552951&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207682%26dopt%3DAbstract Conclusions. While our results indicate that HIF-2 is not directly involved in hepcidin repression, they highlight the contribution of hepatic HIF-2 to repress hepcidin through EPO-mediated increased erythropoiesis, a result of potential clinical interest. PMID: 22207682 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5552951 Thu, 29 Dec 2011 05:00:00 +0100 5552951 http://www.medworm.com/index.php?rid=5552950&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207683%26dopt%3DAbstract Conclusions. A negative PET/CT reliably rules out a relapse. The high false positive rate, however, is an important limitation and a confirmatory biopsy is mandatory for the diagnosis of a relapse. With no proven survival benefit for patients with a pre-clinically diagnosed relapse, the high costs and low positive predictive value make PET/CT unfit for routine surveillance of Hodgkin lymphoma patients. PMID: 22207683 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5552950 Thu, 29 Dec 2011 05:00:00 +0100 5552950 http://www.medworm.com/index.php?rid=5552949&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207684%26dopt%3DAbstract Conclusions. Our results provide strong evidence that NAMPT and SIRT2 participate in the aberrant proliferation and survival of leukemic cells, and suggest that the AKT/GSK-3β/β-catenin pathway is a target in FK866- and AC93253-induced inhibition of leukemia cell proliferation. PMID: 22207684 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5552949 Thu, 29 Dec 2011 05:00:00 +0100 5552949 http://www.medworm.com/index.php?rid=5552948&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207685%26dopt%3DAbstract Conclusions. Analysis of multiple myeloma immunoglobulin repertoire does not support a pathogenetic role for antigen selection in this tumor. PMID: 22207685 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5552948 Thu, 29 Dec 2011 05:00:00 +0100 5552948 http://www.medworm.com/index.php?rid=5552947&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207686%26dopt%3DAbstract ConclusionOur study supports the notion that endothelial cells are major players in chronic lymphocytic leukemia microenvironment. Adhesion to endothelium strongly sustains survival, protects from drug-induced apoptosis and widely modifies gene expression profile of leukemic cells. PMID: 22207686 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5552947 Thu, 29 Dec 2011 05:00:00 +0100 5552947 http://www.medworm.com/index.php?rid=5552946&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207687%26dopt%3DAbstract Conclusions. Our data demonstrate that glycoproteins B and E are major targets of varicella-zoster virus specific CD4+ and CD8+ T-cell reconstitution occurring during herpes zoster after allogeneic stem-cell transplantation. Varicella-zoster virus glycoproteins B and E might form the basis for novel nonhazardous zoster subunit vaccines suitable for immunocompromised transplant patients. PMID: 22207687 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5552946 Thu, 29 Dec 2011 05:00:00 +0100 5552946 http://www.medworm.com/index.php?rid=5552945&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207688%26dopt%3DAbstract Authors: Bi Y, Zeng N, Chanudet E, Huang Y, Hamoudi RA, Liu H, Dong G, Watkins AJ, Ley SC, Zhou L, Chen R, Zhu X, Du MQ Abstract Recent studies showed A20 inactivation by deletion, mutation and promoter methylation in ocular adnexal mucosa-associated lymphoid tissue lymphoma. However, the incidences of A20 abnormalities and their clinical impacts are largely unknown. It is also unknown whether ABIN-1 and ABIN-2, the components of the A20 NF-kB inhibitor complex, are inactivated by genetic changes in ocular adnexal mucosa-associated lymphoid tissue lymphoma. A total of 105 cases were investigated for A20 mutation/deletion, ABIN-1/2 mutation, MALT1 and IGH involved translocation. Somatic mutation was seen frequently in A20 (28.6%), but rarely in ABIN-1 (1%) and ABIN-2 (1%). A20 muta... Haematologica journals http://www.medworm.com/rss/comments.php?id=5552945 Thu, 29 Dec 2011 05:00:00 +0100 5552945 http://www.medworm.com/index.php?rid=5552944&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207689%26dopt%3DAbstract Conclusions: We postulate that natural mutations of cysteines involved in the formation of interchain disulfide bonds do not affect the storage in Weibel-Palade bodies and secretion of von Willebrand factor, whereas mutations of cysteines forming intrachain disulfide bonds will lead to reduced von Willebrand factor storage and secretion due to ER retention. PMID: 22207689 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5552944 Thu, 29 Dec 2011 05:00:00 +0100 5552944 http://www.medworm.com/index.php?rid=5552943&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207690%26dopt%3DAbstract Conclusions. High dose imatinib leads to superior molecular responses in patients with low OCT-1 activity. In this group trough imatinib levels may define a group with inferior outcomes. For high OCT-1 activity patients higher imatinib dosing or monitoring of imatinib trough levels were not found to be of significant clinical value. Hence OCT-1 activity determined prior to the start of therapy in newly diagnosed CML patients provides a valuable prognostic tool to determine the optimal up-front dose of imatinib in newly diagnosed chronic phase chronic myeloid leukaemia patients. PMID: 22207690 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5552943 Thu, 29 Dec 2011 05:00:00 +0100 5552943 http://www.medworm.com/index.php?rid=5552942&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22207691%26dopt%3DAbstract Authors: Del Giudice I, Rossi D, Chiaretti S, Marinelli M, Tavolaro S, Gabrielli S, Laurenti L, Marasca R, Rasi S, Fangazio M, Guarini A, Gaidano G, Foa' R Abstract Trisomy 12, the third most frequent chromosomal aberration in chronic lymphocytic leukemia (CLL), confers an intermediate prognosis. In our cohort of 104 untreated patients carrying +12, NOTCH1 mutations occurred in 24% of cases and were associated to unmutated IGHV genes (p=.003) and +12 as a sole cytogenetic abnormality (p=.008). NOTCH1 mutations in +12 CLL associated with a ~2.4 fold increase in the death risk, a significant survival shortening (p<.001) and proved an independent predictor of survival in multivariate analysis. Analogous to +12 CLL with TP53 disruption or del(11q), NOTCH1 mutations in +12 CLL confer... Haematologica journals http://www.medworm.com/rss/comments.php?id=5552942 Thu, 29 Dec 2011 05:00:00 +0100 5552942 http://www.medworm.com/index.php?rid=5539108&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180419%26dopt%3DAbstract Conclusions. Polymorphisms in the interleukin-28B are associated with the control of hepatitis C virus infection in Thalassemia Major patients, and the knowledge of allelic patterns has a relevant role in determining the prognosis and therapeutic management. PMID: 22180419 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539108 Fri, 16 Dec 2011 05:00:00 +0100 5539108 http://www.medworm.com/index.php?rid=5539107&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180420%26dopt%3DAbstract Conclusions:Thrombolytic agents successfully clear central venous catheter occlusions in most cases. Newer agents may act more rapidly and effectively than currently utilized therapies, but randomized studies with direct comparisons of these agents are needed to determine optimal management for catheter obstruction. PMID: 22180420 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539107 Fri, 16 Dec 2011 05:00:00 +0100 5539107 http://www.medworm.com/index.php?rid=5539106&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180421%26dopt%3DAbstract Authors: Kristinsson SY, Tang M, Pfeiffer RM, Bjorkholm M, Goldin LR, Blimark C, Mellqvist UH, Wahlin A, Turesson I, Landgren O Abstract No comprehensive evaluation has been conducted to assess the risk of viral and bacterial infections among patients with monoclonal gammopathy of undetermined significance. Using population-based data from Sweden, we estimated risk of infections among 5,326 MGUS patients compared to 20,161 matched controls. Patients with monoclonal gammopathy of undetermined significance had a 2-fold increased risk (P<0.05) of developing any infection at 5 and 10 years of follow-up. More specifically, patients with monoclonal gammopathy of undetermined significance had an increased risk (P<0.05) of bacterial infections (pneumonia, osteomyelitis, septicemia, p... Haematologica journals http://www.medworm.com/rss/comments.php?id=5539106 Fri, 16 Dec 2011 05:00:00 +0100 5539106 http://www.medworm.com/index.php?rid=5539105&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180422%26dopt%3DAbstract Conclusions:Maternal and fetal status as well as dietary iron is important in regulating iron transfer across placenta. Maternal Hfe regulates iron transfer by altering gene expression in the placenta. Fetal Hfe is important in regulating placental iron transfer by modulating fetal liver hepcidin expression. PMID: 22180422 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539105 Fri, 16 Dec 2011 05:00:00 +0100 5539105 http://www.medworm.com/index.php?rid=5539104&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180423%26dopt%3DAbstract Conclusions. Our data show that subcutaneous MALT-lymphoma involvement is a rare event in patients with a priori non-cutaneous extranodal marginal zone lymphoma. However, it seems to be almost exclusively associated with MALT-lymphoma of the ocular adnexa and the breast, suggesting as yet undefined interactions between potentially embryologically related organ systems. PMID: 22180423 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539104 Fri, 16 Dec 2011 05:00:00 +0100 5539104 http://www.medworm.com/index.php?rid=5539103&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180424%26dopt%3DAbstract This article will review the data available on acute myeloid leukemia developing in patients with autoimmune diseases. Possible leukemogeneic mechanisms in these patients as well as evidence supporting the association of their primary immunosuppressive status and their exposure to specific therapies will be reviewed as well. This review also supports the idea that it may be misleading to label leukemias that develop in patients with autoimmune diseases who are exposed to cytotoxic agents as therapy related leukemias. A better understanding of the molecular defects in autoimmune disease patients who develop acute leukemia will lead to a thorough appreciation of the association between these two diseases entities. PMID: 22180424 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539103 Fri, 16 Dec 2011 05:00:00 +0100 5539103 http://www.medworm.com/index.php?rid=5539102&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180425%26dopt%3DAbstract Conclusions. Cyclophosphamide and antithymocyte globulin is an effective conditioning regimen for patients with severe aplastic anemia and is associated with low treatment-related mortality. Long-term complications include avascular necrosis and endocrine dysfunction.Key words: Hematopoietic stem cell transplantation, severe aplastic anemia, long-term outcomes. PMID: 22180425 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539102 Fri, 16 Dec 2011 05:00:00 +0100 5539102 http://www.medworm.com/index.php?rid=5539101&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180426%26dopt%3DAbstract Conclusions. Improvement of ex vivo fludarabine sensitivity in chronic lymphocytic leukemia cells is associated with increased hCNT3 activity after all-trans-retinoic acid treatment. PMID: 22180426 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539101 Fri, 16 Dec 2011 05:00:00 +0100 5539101 http://www.medworm.com/index.php?rid=5539100&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180427%26dopt%3DAbstract ConclusionThe widely used assay chosen for this study detects both endogenous non-transferrin bound iron and the iron complexes of deferiprone. The week 1 increments reflect chelatable iron derived both from liver stores and from red cell catabolism. These increments correlate with urine iron excretion and the change in liver iron concentration over the subsequent year thus predicting response to deferiprone-containing regimes.This clinical study has been registered at clinical.trials.gov with the number NCT00350662. PMID: 22180427 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539100 Fri, 16 Dec 2011 05:00:00 +0100 5539100 http://www.medworm.com/index.php?rid=5539099&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180428%26dopt%3DAbstract Conclusions. Our studies indicate that macromolecule-associated non-transferrin-bound iron can initially gain access into various cells via endocytic pathways, followed by iron translocation to the cytosol. Endocytic uptake of plasma non-transferrin-bound iron is a possible mechanism that can contribute to iron loading of cell types engaged in bulk/adsorptive endocytosis, highlighting the importance of its prevention by iron chelation. PMID: 22180428 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539099 Fri, 16 Dec 2011 05:00:00 +0100 5539099 http://www.medworm.com/index.php?rid=5539098&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180429%26dopt%3DAbstract Conclusions. This new family of mobilizing agent could alone or in combination with G-CSF and/or AMD3100 mobilize a high number of hematopoietic stem cells that can maintain long-term hematopoiesis. These results strengthen the role of heparan sulfates in the retention of hematopoietic stem cells in bone marrow and support the use of small glyco-drugs based on heparan sulfate in combination with G-CSF and AMD3100 to improve high stem cell mobilization, particularly in a human therapeutic prospect. PMID: 22180429 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539098 Fri, 16 Dec 2011 05:00:00 +0100 5539098 http://www.medworm.com/index.php?rid=5539097&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180430%26dopt%3DAbstract Conclusions. Although functional studies show an oncogenic role of the hepatocyte growth factor/c-Met signaling pathway in cell cycle progression, expression of c-Met in tumor cells of classical Hodgkin lymphoma patients strongly correlates with favorable prognosis in two independent cohorts. PMID: 22180430 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539097 Fri, 16 Dec 2011 05:00:00 +0100 5539097 http://www.medworm.com/index.php?rid=5539096&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180431%26dopt%3DAbstract Authors: Yanada M, Kurosawa S, Yamaguchi T, Yamashita T, Moriuchi Y, Ago H, Takeuchi J, Nakamae H, Taguchi J, Sakura T, Takamatsu Y, Waki F, Yokoyama H, Watanabe M, Emi N, Fukuda T Abstract To evaluate the prognostic impact of monosomal karyotype on post-remission outcome in acute myeloid leukemia, we retrospectively analyzed 2,099 patients who had achieved complete remission. Monosomal karyotype was noted in 73 patients (4%). Of them, the probability of overall survival from first complete remission was 14% at 4 years, which was significantly inferior to that in patients without monosomal karyotype, primarily due to a high relapse rate of 86%. Monosomal karyotype remained significantly associated with worse overall survival among patients with unfavorable cytogenetics or complex k... Haematologica journals http://www.medworm.com/rss/comments.php?id=5539096 Fri, 16 Dec 2011 05:00:00 +0100 5539096 http://www.medworm.com/index.php?rid=5539095&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180432%26dopt%3DAbstract Conclusions:Our data suggest that the combination of Bryostatin1 with antibodies directed against CD22 is a potent drug combination for the treatment of low and high-grade B-cell lymphoma. PMID: 22180432 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539095 Fri, 16 Dec 2011 05:00:00 +0100 5539095 http://www.medworm.com/index.php?rid=5539094&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180433%26dopt%3DAbstract This study shows for the first time a link between homozygous MPL mutations and familial aplastic anaemia. It also highlights the important role of MPL in trilineage haematopoiesis. PMID: 22180433 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539094 Fri, 16 Dec 2011 05:00:00 +0100 5539094 http://www.medworm.com/index.php?rid=5539093&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180434%26dopt%3DAbstract ConclusionPatients who require a second salvage regimen to achieve disease control prior to autologous stem cell transplantation have a relatively poor outcome and should be considered for alternative treatment strategies. PMID: 22180434 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539093 Fri, 16 Dec 2011 05:00:00 +0100 5539093 http://www.medworm.com/index.php?rid=5539092&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180435%26dopt%3DAbstract Authors: Takahashi N, Kyo T, Maeda Y, Sugihara T, Usuki K, Kawaguchi T, Usui N, Okamoto S, Ohe Y, Ohtake S, Kitamura K, Yamamoto M, Teshima H, Motoji T, Tamaki T, Sawada K, Ohyashiki K Abstract It was recently recognized that some chronic myeloid leukemia patients with a complete molecular response could sustain that response after discontinuation of imatinib. To characterize the clinical outcomes and profiles of chronic phase chronic myeloid leukemia patients who could discontinue imatinib, we conducted a nationwide survey in Japan. Among 3,242 imatinib-treated chronic myeloid leukemia patients, 50 were identified who discontinued imatinib for at least 6 months; of those, 43 were analyzed. Molecular recurrence was detected in 19 patients, and a complete molecular response rate was... Haematologica journals http://www.medworm.com/rss/comments.php?id=5539092 Fri, 16 Dec 2011 05:00:00 +0100 5539092 http://www.medworm.com/index.php?rid=5539091&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180436%26dopt%3DAbstract We report two asymptomatic homozygotes for the nonsense p.R462X mutation affecting the carboxy-terminus of coagulation factor VII (FVII, 466 aminoacids). FVII levels of 3-5% and 2.7±0,4% were found in Prothrombin Time-based and activated factor X (FXa) generation assays with human thromboplastins. Noticeably, FVII antigen levels were barely detectable (0.7±0,2%), which suggested a gain-of-function effect. This effect was more pronounced with bovine thromboplastin (4.8±0,9%) and disappeared with rabbit thromboplastin (0.7±0,2%), thus pointing toward an influence of the mutation on Tissue Factor/FVII interactions. Whereas the recombinant rFVII-462X variant confirmed an increased specific activity (≈400%), a panel of nonsense (p.P466X, p.F465X, p.P464X, p.A463X) and missense (p.R462A, p... Haematologica journals http://www.medworm.com/rss/comments.php?id=5539091 Fri, 16 Dec 2011 05:00:00 +0100 5539091 http://www.medworm.com/index.php?rid=5539090&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22180437%26dopt%3DAbstract Conclusions. The poor reliability of the 10% cut off was partly related to the proximity of the current criteria to the observed cut-off mean values of the normal population. These findings question the current WHO threshold of ≥ 10% necessary for the percentage of cells manifesting dysplasia as significant and suggest that either a higher threshold would be more appropriate or different thresholds should be set for each lineage. PMID: 22180437 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539090 Fri, 16 Dec 2011 05:00:00 +0100 5539090 http://www.medworm.com/index.php?rid=5539112&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22147769%26dopt%3DAbstract Authors: Schwanbeck R, Just U PMID: 22147769 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539112 Thu, 01 Dec 2011 05:00:00 +0100 5539112 http://www.medworm.com/index.php?rid=5539111&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22147770%26dopt%3DAbstract Authors: Carella AM, Goldman JM, Martinelli G, Melo JV, Perrotti D PMID: 22147770 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539111 Thu, 01 Dec 2011 05:00:00 +0100 5539111 http://www.medworm.com/index.php?rid=5539110&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22147771%26dopt%3DAbstract Authors: Schulze H, Gaedicke G PMID: 22147771 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539110 Thu, 01 Dec 2011 05:00:00 +0100 5539110 http://www.medworm.com/index.php?rid=5539109&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22147772%26dopt%3DAbstract Authors: Cazzola M PMID: 22147772 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5539109 Thu, 01 Dec 2011 05:00:00 +0100 5539109 http://www.medworm.com/index.php?rid=5480956&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133767%26dopt%3DAbstract Conclusion: Patients with chronic idiopathic neutropenia display age-inappropriate telomereshortening of peripheral blood cells and low telomerase activity in peripheral blood mononuclear cells. A compensatory increased proliferation of bone marrow hematopoietic progenitor cells in association with lymphocyte replicative exhaustion probably account for these abnormalities. PMID: 22133767 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480956 Thu, 01 Dec 2011 05:00:00 +0100 5480956 http://www.medworm.com/index.php?rid=5480955&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133768%26dopt%3DAbstract CONCLUSIONS. These data indicate that risk of in-hospital venous thromboembolism in children with this risk factor combination may exceed that of hospitalized adults in whom prophylactic anticoagulation is indicated. Substantiation of these findings via multicenter studies could provide the basis for future risk-stratified randomized control trials of pediatric venous thromboembolism prevention. PMID: 22133768 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480955 Thu, 01 Dec 2011 05:00:00 +0100 5480955 http://www.medworm.com/index.php?rid=5480954&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133769%26dopt%3DAbstract Authors: Blombery P, Wong SQ, Hewitt CA, Dobrovic A, Maxwell EL, Juneja S, Grigoriadis G, Westerman DA Abstract Hairy cell leukemia has been shown to be strongly associated with the BRAF V600E mutation. We screened 59 unenriched archived bone marrow aspirate and peripheral blood samples from 51 patients with hairy cell leukemia using high resolution melting analysis and confirmatory Sanger sequencing. The BRAF V600E mutation was detected in 38 samples (from 36 patients). The BRAF V600E mutation was detected in all samples with disease involvement above the limit of sensitivity of the techniques used. 33/34 samples from other hematological malignancies were negative for BRAF mutations. A BRAF K601E mutation was detected in a patient with splenic marginal zone lymphoma. Our data supp... Haematologica journals http://www.medworm.com/rss/comments.php?id=5480954 Thu, 01 Dec 2011 05:00:00 +0100 5480954 http://www.medworm.com/index.php?rid=5480953&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133770%26dopt%3DAbstract Conclusions. This combination therapy appears to be an efficacious and safe treatment for steroid-refractory chronic graft-versus-host disease. Longer follow-up is necessary in this study in order to determine the durability of response and survival. (ClinicalTrials.gov: NCT01042509). PMID: 22133770 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480953 Thu, 01 Dec 2011 05:00:00 +0100 5480953 http://www.medworm.com/index.php?rid=5480952&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133771%26dopt%3DAbstract Authors: Walter RB, Medeiros BC, Powell BL, Schiffer CA, Appelbaum FR, Estey EH Abstract Histone deacetylase inhibitors such as vorinostat enhance gemtuzumab ozogamicin efficacy in vitro. We therefore investigated vorinostat+gemtuzumab ozogamicin for adults ≥60 years with untreated acute myeloid leukemia. We stratified patients into 2 groups (group 1: patients aged ≥70 years and performance status 2-3; group 2: aged 60-69 years with performance status 0-3 or aged ≥70 years and performance status 0-1), and monitored responses separately in group 2 patients with normal or favorable cytogenetics (group 2A) and other cytogenetics (group 2B). Among 31 patients, 6 (19.4%) achieved complete remission, and 1 (3.2%) achieved complete remission with incomplete platelet recovery; these ... Haematologica journals http://www.medworm.com/rss/comments.php?id=5480952 Thu, 01 Dec 2011 05:00:00 +0100 5480952 http://www.medworm.com/index.php?rid=5480951&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133772%26dopt%3DAbstract Conclusions: These results provide the first confirmation by a multi-institutional group that DA-EPOCH-R provides high durable remissions in diffuse large B-cell lymphoma and is effective in both germinal center and non-germinal center B-cell subtypes. The trial was registered at ClinicalTrials.Gov (NCT00032019). PMID: 22133772 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480951 Thu, 01 Dec 2011 05:00:00 +0100 5480951 http://www.medworm.com/index.php?rid=5480950&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133773%26dopt%3DAbstract Conclusions. Purine analogs, particularly combined with cyclophosphamide, significantly improve progression free survival but not survival. Some groups, such as the elderly, may not see the same benefits and maximising doses may be important for all treatments, including chlorambucil. Longer follow-up, consistent definitions and detailed reporting of trials should be encouraged. PMID: 22133773 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480950 Thu, 01 Dec 2011 05:00:00 +0100 5480950 http://www.medworm.com/index.php?rid=5480949&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133774%26dopt%3DAbstract Authors: Kunicki TJ, Williams SA, Diaz D, Farndale RW, Nugent DJ Abstract A single nucleotide polymorphism in the integrin α2 gene ITGA2 (rs1801106; G1600A) creates the non-conservative amino acid substitution E534K, the basis of the human platelet alloantigen system HPA-5, yet HPA-5 alleles do not influence binding of α2β1 to its primary ligand collagen I, and the effect of HPA-5 on platelet function has not been determined. We used a direct platelet adhesion assay to evaluate whether differential inheritance of HPA-5 alleles influences platelet adhesion to collagen I or an alternative ligand, decorin. Platelets from donors bearing one or more minor allele HPA-5b exhibited attenuated adhesion to purified decorin but not collagen I. Adhesion to decorin was significantly inhibite... Haematologica journals http://www.medworm.com/rss/comments.php?id=5480949 Thu, 01 Dec 2011 05:00:00 +0100 5480949 http://www.medworm.com/index.php?rid=5480948&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133775%26dopt%3DAbstract Authors: Macpherson L, Monypenny J, Blundell MP, Cory GO, Tome'-Garcia J, Thrasher AJ, Jones GE, Calle Y Abstract Podosomes are actin-based adhesions involved in migration of cells that have to cross tissue boundaries such as myeloid cells. The Wiskott Aldrich Syndrome Protein regulates de novo actin polymerisation during podosome formation and it is cleaved by the protease calpain during podosome disassembly. The mechanisms that may induce the Wiskott Aldrich Syndrome Protein cleavage by calpain remain undetermined. We now report that in myeloid cells, tyrosine phosphorylation of the Wiskott Aldrich Syndrome Protein-tyrosine291 (Human)/tyrosine293 (mouse) not only enhances Wiskott Aldrich Syndrome Protein-mediated actin polymerisation but also promotes its calpain-dependent degrad... Haematologica journals http://www.medworm.com/rss/comments.php?id=5480948 Thu, 01 Dec 2011 05:00:00 +0100 5480948 http://www.medworm.com/index.php?rid=5480947&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133776%26dopt%3DAbstract Conclusions. This was the first randomized controlled trial to Although thalidomide was not superior to dexamethasone in this randomized trial, demonstrate that thalidomide monotherapy may be considered is an effective salvage therapy option for relapsed/refractory multiple myeloma, particularly in patients with good prognosis and those who have received 2-3 prior therapies. The recommended starting dose of thalidomide monotherapy is 400 mg/day, which can be rapidly reduced for patients who cannot tolerate treatment.(Clinical trial registration number: NCT00452569). PMID: 22133776 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480947 Thu, 01 Dec 2011 05:00:00 +0100 5480947 http://www.medworm.com/index.php?rid=5480946&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133777%26dopt%3DAbstract Authors: Milojkovic D, Ibrahim A, Reid A, Foroni L, Apperley J, Marin D Abstract In this paper we show that dasatinib can be safely combined with conventional chemotherapy and although this approach should be tested in a larger number of patients the combination seems to induce deep remissions in patients with CML in blastic phase allowing for further therapeutic strategies to enable a continuing response. PMID: 22133777 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480946 Thu, 01 Dec 2011 05:00:00 +0100 5480946 http://www.medworm.com/index.php?rid=5480945&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133778%26dopt%3DAbstract ConclusionsThus, both patient and donor contribute to the changes in cytokine levels.The study reported here was performed using plasma samples drawn from patients enrolled in the ClinicalTrials.gov-registered trials NCT00467961 and NCT00378534. PMID: 22133778 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480945 Thu, 01 Dec 2011 05:00:00 +0100 5480945 http://www.medworm.com/index.php?rid=5480944&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133779%26dopt%3DAbstract Conclusions. These findings suggest that SET overexpression represents a key mechanism to inhibit PP2A in acute myeloid leukemia, and that EVI1 overexpression contributes to deregulate SET. Furthermore, SET overexpression is associated with poor outcome in acute myeloid leukemia, and it can be used to identify a subgroup of patients that could benefit from future treatments based on PP2A activators. PMID: 22133779 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480944 Thu, 01 Dec 2011 05:00:00 +0100 5480944 http://www.medworm.com/index.php?rid=5480943&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133780%26dopt%3DAbstract Conclusions. Although it is associated with a significant but manageable risk of bleeding, systemic thrombolysis is a highly effective treatment in order to reverse venous thromboses in patients with paroxysmal nocturnal hemoglobinuria. PMID: 22133780 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480943 Thu, 01 Dec 2011 05:00:00 +0100 5480943 http://www.medworm.com/index.php?rid=5480942&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22133781%26dopt%3DAbstract Conclusions. This assay could help to define a standard for the quantitation of anti-HPA antibodies. Finally, this report demonstrates that peptide aptamers can potentially detect a variety of biomarkers in body fluids, being thus of particular interest for diagnostic purposes. PMID: 22133781 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5480942 Thu, 01 Dec 2011 05:00:00 +0100 5480942 http://www.medworm.com/index.php?rid=5447506&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102700%26dopt%3DAbstract Conclusions. Activated endothelial cells show significant phagocytosis of phosphatidylserine-exposing and rigid red blood cells under both static and flow conditions, resulting in cytotoxicity. This results in a certain degree of cytotoxicity. We postulate that activated endothelial cells play a role in RBC clearance in vivo, which contribute to the vasopathological effects as seen, for instance, in sickle cell disease. PMID: 22102700 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5447506 Fri, 18 Nov 2011 05:00:00 +0100 5447506 http://www.medworm.com/index.php?rid=5447505&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102701%26dopt%3DAbstract Conclusions. According to the statistical design of the current study, neither of the two infliximab dose schedules tested showed sufficient activity as a single agent in this cohort of unselected patients with early myelodysplastic syndrome. (ClinicalTrials.gov number, NCT00074074). PMID: 22102701 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5447505 Fri, 18 Nov 2011 05:00:00 +0100 5447505 http://www.medworm.com/index.php?rid=5447504&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102702%26dopt%3DAbstract Conclusions. Despite the link between use of erythropoiesis stimulating agents and thrombosis among patients with solid tumors, this study provides evidence that their safety profile may be different among patients with myelodysplastic syndromes. PMID: 22102702 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5447504 Fri, 18 Nov 2011 05:00:00 +0100 5447504 http://www.medworm.com/index.php?rid=5447503&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102703%26dopt%3DAbstract Authors: Yan Y, Huang Y, Watkins AJ, Kocialkowski S, Zeng N, Hamoudi RA, Isaacson PG, de Leval L, Wotherspoon A, Du MQ Abstract The genetics and pathogenesis of splenic marginal zone lymphoma are poorly understood. The lymphoma lacks chromosome translocation, and ~30% of cases are featured by 7q deletion, but the gene targeted by the deletion is unknown. A recent study showed inactivation of A20, a 'global' NF-kB negative regulator, in 1 of 12 splenic marginal zone lymphoma. To investigate further whether deregulation of the NF-kB pathway plays a role in the pathogenesis of splenic marginal zone lymphoma, we screened several NF-kB regulators for genetic changes by PCR and sequencing. Somatic mutations were found in A20 (6/46=13%), MYD88 (6/46=13%), CARD11 (3/34=8.8%), but not in C... Haematologica journals http://www.medworm.com/rss/comments.php?id=5447503 Fri, 18 Nov 2011 05:00:00 +0100 5447503 http://www.medworm.com/index.php?rid=5447502&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102704%26dopt%3DAbstract Authors: Rio-Machin A, Menezes J, Maiques-Diaz A, Agirre X, Ferreira BI, Acquadro F, Rodriguez-Perales S, Arribalzaga Juaristi K, Alvarez S, Cigudosa JC Abstract AbstractThe disruption of RUNX1 function is one of the main mechanisms of disease observed on hematopoietic malignancies and the description of novel genetic events that leads to a RUNX1 loss of function has been accelerated with the development of the genomic technologies. Here we describe the molecular characterization of a new t(4;21)(q21;q22) in a "de novo" myelodysplastic syndrome that resulted in the deletion of RUNX1 gene. We demonstrated by quantitative real-time RT-PCR, an almost complete depletion of the expression of RUNX1 gene in our t(4;21) case compared with CD34+ cells that was independent of mutation or DNA... Haematologica journals http://www.medworm.com/rss/comments.php?id=5447502 Fri, 18 Nov 2011 05:00:00 +0100 5447502 http://www.medworm.com/index.php?rid=5447501&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102705%26dopt%3DAbstract Authors: Gallienne AE, Dreau HM, Schuh A, Old J, Henderson S Abstract We investigated whether mutations in the KLF1 gene are associated with increased Hb F levels in ethnically diverse patients referred to our laboratory for haemoglobinopathy investigation. Deleterious KLF1 mutations were identified in 11 out of 131 adult samples with an elevated Hb F level (1.5-25.0%).Eleven different mutations were identified, 9 of which were previously un-reported. KLF1 mutations were not identified in a matched cohort of 121 samples with normal Hb F levels (<1.0%). A further novel KLF1 mutation was also found in a sickle cell disease patient with a Hb F level of 20.3% who had a particularly mild phenotype. Our results indicate KLF1 mutations could make a significant contribution to Hb F vari... Haematologica journals http://www.medworm.com/rss/comments.php?id=5447501 Fri, 18 Nov 2011 05:00:00 +0100 5447501 http://www.medworm.com/index.php?rid=5447500&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102706%26dopt%3DAbstract Conclusions. Posaconazole primary antifungal prophylaxis during front-line chemotherapy was effective in preventing invasive fungal diseases in a 'real life' scenario of acute myeloid leukemia patients, resulted in an early but transitory survival advantage in younger patients and was associated to economical advantages. PMID: 22102706 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5447500 Fri, 18 Nov 2011 05:00:00 +0100 5447500 http://www.medworm.com/index.php?rid=5447499&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102707%26dopt%3DAbstract Authors: Xue SL, Li JL, Zou JY, Su J, Chen SN, Wu DP Abstract Severe congenital neutropenia (SCN) could exhibit different modes of inheritance from a genetic perspective, including autosomal dominant, autosomal recessive, X-linked forms and a sporadic one without any hints regarding family history. Investigations in molecular etiology have revealed some specific genes mutation that could account for the different types of SCN respectively. Homozygous HAX1 mutations had been identified in autosomal recessive SCN (Kostmann disease) previously. However, compound heterozygous HAX1 mutations were also confirmed in autosomal recessive SCN by subsequent research. Here we described another patient, also the first Chinese patient, with autosomal recessive SCN carrying a novel compound heter... Haematologica journals http://www.medworm.com/rss/comments.php?id=5447499 Fri, 18 Nov 2011 05:00:00 +0100 5447499 http://www.medworm.com/index.php?rid=5447498&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102708%26dopt%3DAbstract Conclusion Generally, hematologic response was not accompanied by molecular response. Therefore, a quantitative change in %V617F is not required for clinical response in patients with polycythemia vera. PMID: 22102708 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5447498 Fri, 18 Nov 2011 05:00:00 +0100 5447498 http://www.medworm.com/index.php?rid=5447497&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102709%26dopt%3DAbstract Conclusion: Detection of minimal residual disease following induction immunochemotherapy was an independent predictor of time to progression following immunochemotherapy and autologous stem cell transplant for mantle cell lymphoma. PMID: 22102709 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5447497 Fri, 18 Nov 2011 05:00:00 +0100 5447497 http://www.medworm.com/index.php?rid=5447496&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102710%26dopt%3DAbstract Conclusions: Follicular lymphoma is characterized by a unique microRNA signature, containing a subset of miRs whose expression correlate with response to chemotherapy. MiR-20a/b and miR-194 target CDKN1A and SOCS2 in follicular lymphoma, potentially contributing to tumor cell proliferation and survival. PMID: 22102710 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5447496 Fri, 18 Nov 2011 05:00:00 +0100 5447496 http://www.medworm.com/index.php?rid=5447495&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102711%26dopt%3DAbstract Conclusions. We present a new approach to identify spike genes and their link to patients' survival. PMID: 22102711 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5447495 Fri, 18 Nov 2011 05:00:00 +0100 5447495 http://www.medworm.com/index.php?rid=5447494&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22102712%26dopt%3DAbstract Authors: Passarello C, Giambona A, Cannata M, Vinciguerra M, Renda D, Maggio A Abstract - PMID: 22102712 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5447494 Fri, 18 Nov 2011 05:00:00 +0100 5447494 http://www.medworm.com/index.php?rid=5428295&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058194%26dopt%3DAbstract Conclusion: Outcome of hypocellular acute myeloid leukemia does not differ from that of non-hypocellular acute myeloid leukemia. PMID: 22058194 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428295 Fri, 04 Nov 2011 04:00:00 +0100 5428295 http://www.medworm.com/index.php?rid=5428294&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058195%26dopt%3DAbstract Conclusions. Our data provide the first evidence of a differential response of p185BCR/ABL- and p210BCR/ABL-transformed cells to the allosteric inhibition by GNF-2, which is of importance for the treatment of Philadelphia chromosome positive acute lymphatic leukemia patients. PMID: 22058195 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428294 Fri, 04 Nov 2011 04:00:00 +0100 5428294 http://www.medworm.com/index.php?rid=5428293&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058196%26dopt%3DAbstract In conclusion, ATP-binding cassette transporters' activity remains an independent prognostic factor, and could be useful for treatment decisions in patients with no nucleophosmin mutation and no fms-like tyrosine kinase 3 duplication. PMID: 22058196 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428293 Fri, 04 Nov 2011 04:00:00 +0100 5428293 http://www.medworm.com/index.php?rid=5428292&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058197%26dopt%3DAbstract Conclusion: B cells diverge from one chronic lymphocytic leukemia patient to another with respect to their membrane lipid organization. In practice, given the relevance of the membrane lipid distribution to the efficacy of biotherapies, such observations are of potential importance. PMID: 22058197 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428292 Fri, 04 Nov 2011 04:00:00 +0100 5428292 http://www.medworm.com/index.php?rid=5428291&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058198%26dopt%3DAbstract Authors: Ananda-Rajah MR, Grigg A, Downey MT, Bajel A, Spelman T, Cheng AC, Thursky KA, Vincent J, Slavin MA Abstract Post-induction aplasia for acute myeloid leukaemia (AML)/myelodysplastic syndrome (MDS) is a high-risk period for invasive fungal diseases (IFDs). The effectiveness of fluconazole, itraconazole solution, voriconazole and posaconazole prophylaxis used consecutively from December 1998-January 2010 in patients with AML/MDS undergoing remission-induction chemotherapy was retrospectively evaluated. A total of 216 consecutive patients received 573 prophylaxis courses. Breakthrough-IFD incidence in fluconazole, itraconazole, voriconazole, posaconazole recipients was 25%, 16%, 14% and 3% respectively. Voriconazole/posconazole versus fluconazole/itraconazole combined was ass... Haematologica journals http://www.medworm.com/rss/comments.php?id=5428291 Fri, 04 Nov 2011 04:00:00 +0100 5428291 http://www.medworm.com/index.php?rid=5428290&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058199%26dopt%3DAbstract Conclusion: Our data show that hydrogel-based cultures of mesenchymal stromal cells form a functional in vitro niche by mimicking key features of the marrow physiology. PMID: 22058199 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428290 Fri, 04 Nov 2011 04:00:00 +0100 5428290 http://www.medworm.com/index.php?rid=5428289&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058200%26dopt%3DAbstract Conclusions. These results confirm a favorable safety and activity profile of lenalidomide in relapsed/refractory MCL. The contribution of dexamethasone in achieving these results is unclear because of its possible detrimental effect on the immune activation generated by lenalidomide and the higher risk to develop infectious complications. (clinicaltrials.gov identifier: NCT00786851). PMID: 22058200 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428289 Fri, 04 Nov 2011 04:00:00 +0100 5428289 http://www.medworm.com/index.php?rid=5428288&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058201%26dopt%3DAbstract Authors: Homminga I, Vuerhard MJ, Langerak AW, Buijs-Gladdines J, Pieters R, Meijerink JP Abstract Translocation of the LYL1 oncogene are rare in T-cell acute lymphoblastic leukemia, whereas the homologous TAL1 gene is rearranged in ~20% of patients. Previous gene-expression studies have identified an immature T-cell acute lymphoblastic leukemia subgroup with high LYL1 expression in the absence of chromosomal aberrations. Molecular characterization of a t(7;19)(q34;p13) in a pediatric T-cell acute lymphoblastic leukemia patient led to the identification of a translocation between the TRB@ and LYL1 loci. Alike incidental T-cell acute lymphoblastic leukemia cases having synergistic, double translocations affecting TAL1/2 and LMO1/2 oncogenes, this LYL1-translocated patient also had a... Haematologica journals http://www.medworm.com/rss/comments.php?id=5428288 Fri, 04 Nov 2011 04:00:00 +0100 5428288 http://www.medworm.com/index.php?rid=5428287&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058202%26dopt%3DAbstract Conclusions. Based on the high negative predictive value of this diagnostic and therapeutic approach, in persistent febrile neutropenic patients with hematological malignancies or hematopoietic stem cell transplant recipients, it is useful for identifying patients who are not likely to develop invasive fungal infection, therefore do not require antifungal therapy, and with a effectiveness similar to that reported in controlled trials which indicate empirical antifungal therapy universally. PMID: 22058202 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428287 Fri, 04 Nov 2011 04:00:00 +0100 5428287 http://www.medworm.com/index.php?rid=5428286&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058203%26dopt%3DAbstract Conclusions. In situ mantle cell lymphoma lesions are usually an incidental finding with a very indolent behavior. These cases must be distinguished from mantle cell lymphoma with a mantle zone pattern and overt mantle cell lymphoma because they may not require therapeutic intervention. PMID: 22058203 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428286 Fri, 04 Nov 2011 04:00:00 +0100 5428286 http://www.medworm.com/index.php?rid=5428285&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058204%26dopt%3DAbstract Authors: Bissels U, Bosio A, Wagner W Abstract Hematopoiesis is regulated by microRNAs (miRNAs). These small regulatory RNAs are master regulators of developmental processes which modulate expression of several target genes post-transcriptionally. Various miRNAs are up-regulated at specific stages during hematopoietic development and the functional relevance of miRNAs has been proven at many stages of lineage specification. Knock-out of specific miRNAs can produce dramatic phenotypes leading to severe hematopoietic defects. Furthermore, several studies demonstrated that specific miRNAs are differentially expressed in hematopoietic stem cells. However, the emerging picture is extremely complex - due to differences between species, cell type dependent variation in miRNA expression an... Haematologica journals http://www.medworm.com/rss/comments.php?id=5428285 Fri, 04 Nov 2011 04:00:00 +0100 5428285 http://www.medworm.com/index.php?rid=5428284&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058205%26dopt%3DAbstract Conclusions. We demonstrate for the first time that differences in oxygen tension cause selective modification of hematopoietic cell and mesenchymal stromal cell interactions in a co-culture system, thus confirming that this parameter plays a critical role for the interaction between hematopoietic cell and the niche environment. PMID: 22058205 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428284 Fri, 04 Nov 2011 04:00:00 +0100 5428284 http://www.medworm.com/index.php?rid=5428283&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058206%26dopt%3DAbstract Conclusions. These findings suggest that the presence of acute features in patients with chronic graft vs. host disease is a marker for adverse prognosis, greater functional impairment, and higher symptom burden. PMID: 22058206 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428283 Fri, 04 Nov 2011 04:00:00 +0100 5428283 http://www.medworm.com/index.php?rid=5428282&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058207%26dopt%3DAbstract In conclusion, ASXL1 mutations are common mutations in acute myeloid leukemia that are indicative for a poor therapy outcome. PMID: 22058207 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428282 Fri, 04 Nov 2011 04:00:00 +0100 5428282 http://www.medworm.com/index.php?rid=5428281&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058208%26dopt%3DAbstract Conclusions: This study suggests a dual role for epigenetic inactivation of TWIST2 in acute lymphoblastic leukemia, initially through altering cell growth and survival properties and subsequently in increasing the resistance to chemotherapeutic treatment. PMID: 22058208 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428281 Fri, 04 Nov 2011 04:00:00 +0100 5428281 http://www.medworm.com/index.php?rid=5428280&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058209%26dopt%3DAbstract ConclusionsThe cyclophosphamide-thalidomide-dexamethasone regimen showed improved response rates and was not inferior in terms of survival outcomes to the standard infusional regimen of cyclophosphamide-vincristine-doxorubicin-dexamethasone. Based on its oral administration and the reduced incidence of infection and cytopenia, cyclophosphamide-thalidomide-dexamethasone may be considered as an effective induction therapy option for patients with newly diagnosed multiple myeloma. (ISRCTN: 68454111). PMID: 22058209 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428280 Fri, 04 Nov 2011 04:00:00 +0100 5428280 http://www.medworm.com/index.php?rid=5428279&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058210%26dopt%3DAbstract We report here a novel t(X;14)(p11.4;q32.33) identified in 4 lymphoma cases: 2 with a mucosa-associated lymphoid tissue (MALT) lymphoma, 1 with a nodal MZL and 1 with gastric diffuse large B-cell lymphoma. In all cases lymphoma evolved from a previous auto-immune disorder. Fluorescence in situ hybridization (FISH) and molecular studies showed that t(X;14) which is mediated by immunoglobulin heavy chain (IGH) locus targets the G-protein coupled receptor 34 (GPR34) gene, hosted by the CASK gene at Xp11.4. Upregulation of GPR34 mRNA and aberrant expression of GPR34 protein has been demonstrated in 3 presented cases by qRT-PCR and immunohistochemistry, respectively. GPR34 belongs to the largest family of cell surface molecules involved in signal transmission that play important roles in many p... Haematologica journals http://www.medworm.com/rss/comments.php?id=5428279 Fri, 04 Nov 2011 04:00:00 +0100 5428279 http://www.medworm.com/index.php?rid=5428278&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058211%26dopt%3DAbstract Authors: Tomas JF, Giraldo P, Lecumberri R, Nistal S Abstract - PMID: 22058211 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428278 Fri, 04 Nov 2011 04:00:00 +0100 5428278 http://www.medworm.com/index.php?rid=5428277&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058212%26dopt%3DAbstract Conclusions. Our results demonstrate that the recurrent NUP98-CCDC28A is an oncogene that induces a rapid and transplantable myeloid neoplasm in recipient mice. They also provide additional evidence for alternative leukemogenic mechanism for NUP98 oncogenes. PMID: 22058212 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428277 Fri, 04 Nov 2011 04:00:00 +0100 5428277 http://www.medworm.com/index.php?rid=5428276&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058213%26dopt%3DAbstract Conclusions. Each individual test fails to diagnose a portion of cases. The association of EMA-binding and AGLT allowed to identify all the hereditary spherocytosis patients of this series and therefore represents at present an effective diagnostic tool for hereditary spherocytosis also in mild/compensated cases. PMID: 22058213 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428276 Fri, 04 Nov 2011 04:00:00 +0100 5428276 http://www.medworm.com/index.php?rid=5428275&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058214%26dopt%3DAbstract Conclusions:Baseline plasma thymus and activation-regulated chemokine levels correlate with classical Hodgkin lymphoma tumor burden and serial levels correlate with response to treatment in patients with classical Hodgkin lymphoma. PMID: 22058214 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428275 Fri, 04 Nov 2011 04:00:00 +0100 5428275 http://www.medworm.com/index.php?rid=5428274&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058215%26dopt%3DAbstract Conclusions: World Health Organization criteria for discrimination of essential thrombocythemia from prefibrotic primary myelofibrosis are poorly to only moderately reproducible and lead to a higher proportion of non-classifiable myeloproliferative neoplasms than histology alone. PMID: 22058215 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428274 Fri, 04 Nov 2011 04:00:00 +0100 5428274 http://www.medworm.com/index.php?rid=5428273&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058216%26dopt%3DAbstract Authors: Janiszewska H, Bak A, Pilarska M, Heise M, Junkiert-Czarnecka A, Kuliszkiewicz-Janus M, Calbecka M, Jazwiec B, Wolowiec D, Kuliczkowski K, Haus O Abstract Germline mutations of the CHEK2 gene have been reported in some myeloid and lymphoid malignancies, but their impact on development of essential thrombocythemia has not been studied. We detected in 16 out of 106 (15.1%) consecutive patients, newly diagnosed with essential thrombocythemia, one of four analyzed CHEK2 mutations: I157T, 1100delC, IVS2+1G>A or del5395. They were associated with the increased risk of disease (OR=3.8; p=0.002). The median age at ET diagnosis among CHEK2+/JAK2V617F+ patients was seven years lower than among CHEK2-/JAK2V617F+ (52 vs. 59 years; p=0.04), whereas the medians of hematological param... Haematologica journals http://www.medworm.com/rss/comments.php?id=5428273 Fri, 04 Nov 2011 04:00:00 +0100 5428273 http://www.medworm.com/index.php?rid=5428272&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058217%26dopt%3DAbstract Authors: Mannelli F, Gianfaldoni G, Intermesoli T, Cattaneo C, Borlenghi E, Cortelazzo S, Cavattoni I, Pogliani EM, Fumagalli M, Angelucci E, Romani C, Ciceri F, Corti C, Scattolin AM, Cortelezzi A, Mattei D, Audisio E, Spinelli O, Oldani E, Bosi A, Rambaldi A, Bassan R Abstract The prognostic significance of CD20 expression in acute lymphoblastic leukemia has been investigated in children and adults and is still under debate. The aim of our study was to correlate CD20 expression with clinical-biological characteristics and outcome in 172 Philadelphia-negative patients prospectively treated in a multicenter trial introducing the molecular evaluation of minimal residual disease for therapeutic purposes. We considered 20% as the threshold for CD20 positivity. Complete remission rate,... Haematologica journals http://www.medworm.com/rss/comments.php?id=5428272 Fri, 04 Nov 2011 04:00:00 +0100 5428272 http://www.medworm.com/index.php?rid=5428271&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058218%26dopt%3DAbstract Authors: Fabre C, Koscielny S, Mohty M, Fegueux N, Blaise D, Maillard N, Tabrizi R, Michallet M, Socie' G, Yakoub-Agha I, Garban F, Uzunov M, Francois S, Contentin N, Lapusan S, Bourhis JH Abstract Here, we report the French experience on tandem auto-allo-SCT in multiple myeloma on a series of 146 patients treated at 20 centers of the Société Française de Greffe de Moelle et de Thérapie Cellulaire (SFGM-TC) between 1998 and 2010. In this retrospective registry-based study, we examined the long-term outcome and presented a prognostic factor analysis. To be included in our study, the patients had to fully complete the two-step modality of a planned tandem auto-allo transplantation. With a long follow-up of 47.5 months, overall survival and event free survival were 48% and 27% res... Haematologica journals http://www.medworm.com/rss/comments.php?id=5428271 Fri, 04 Nov 2011 04:00:00 +0100 5428271 http://www.medworm.com/index.php?rid=5428270&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058219%26dopt%3DAbstract Conclusions. Decitabine is well tolerated by older, medically non-fit acute myeloid leukemia patients, myelosuppression being the major toxicity. Response rate and overall survival were not adversely influenced by poor-risk cytogenetics or MDS. Because of these encouraging results, randomized studies evaluating single-agent Decitabine versus conventional treatment are warranted. The study is registered with WHO primary registry German Clinical Trials Registry, number DRKS00000069. PMID: 22058219 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428270 Fri, 04 Nov 2011 04:00:00 +0100 5428270 http://www.medworm.com/index.php?rid=5428269&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058220%26dopt%3DAbstract Conclusions: Telomere length by flow fluorescence in situ hybridization is an important diagnostic test for dyskeratosis congenita; age-adjusted values provide a quantitative measure of disease severity (clinical subset, mutated gene, and degree of bone marrow failure). Patients with dyskeratosis congenita have accelerated telomere shortening.Keywords: bone marrow failure, dyskeratosis congenita, telomeres, longitudinal studyTrial registration: This study is registered at www.clinicaltrials.gov (identifier: NCT00027274). PMID: 22058220 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428269 Fri, 04 Nov 2011 04:00:00 +0100 5428269 http://www.medworm.com/index.php?rid=5428268&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058221%26dopt%3DAbstract Conclusions. Altogether, these data demonstrate that interfering with the SDF-1α/CXCR4 axis, by using AMD3100, inhibited chronic lymphocytic leukemia cell trafficking and microenvironment-mediated protective effects. Combining AMD3100 with other drugs may thus represent a promising therapeutic approach to enhance chronic lymphocytic leukemia cell killing. PMID: 22058221 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428268 Fri, 04 Nov 2011 04:00:00 +0100 5428268 http://www.medworm.com/index.php?rid=5428267&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058222%26dopt%3DAbstract Conclusions. Th17 cells are elevated in chronic lymphocytic leukemia patients with better prognostic markers and correlate with longer survival. Furthermore, non-Th17 IL-17A-expressing cells exist in chronic lymphocytic leukemia spleens as maturing granulocytes and mature mast cells, suggesting the microenvironmental milieu in leukemic spleens promotes the recruitment and/or expansion of Th17 and other IL-17-expressing cells. The pathophysiology of Th17 and non-Th17 IL 17A-producing cells in chronic lymphocytic leukemia and their distributions and roles in this disease merit further study. PMID: 22058222 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428267 Fri, 04 Nov 2011 04:00:00 +0100 5428267 http://www.medworm.com/index.php?rid=5428266&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058223%26dopt%3DAbstract Conclusions. Our results show that patients with progressive refractory myeloma do not benefit from autologous transplant, while patients with stable disease have an outcome comparable to those with chemosensitive disease. (ClinicalTrials.gov:NCT00560053). PMID: 22058223 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428266 Fri, 04 Nov 2011 04:00:00 +0100 5428266 http://www.medworm.com/index.php?rid=5428265&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058224%26dopt%3DAbstract Conclusions. Platelet counts after induction treatment may improve treatment stratification for childhood acute lymphoblastic leukemia and be of particular interest to non-minimal residual disease-based trials.ALL-BFM 2000 is registered at: ClinicalTrials.gov: NCT00430118 National Cancer Institute: Protocol ID 68529. PMID: 22058224 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428265 Fri, 04 Nov 2011 04:00:00 +0100 5428265 http://www.medworm.com/index.php?rid=5428264&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058278%26dopt%3DAbstract Authors: Hayflick SJ, Hogarth P PMID: 22058278 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428264 Tue, 01 Nov 2011 04:00:00 +0100 5428264 http://www.medworm.com/index.php?rid=5428263&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058279%26dopt%3DAbstract Authors: Danjou F, Anni F, Galanello R PMID: 22058279 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428263 Tue, 01 Nov 2011 04:00:00 +0100 5428263 http://www.medworm.com/index.php?rid=5428262&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058280%26dopt%3DAbstract Authors: Hermouet S, Vilaine M PMID: 22058280 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428262 Tue, 01 Nov 2011 04:00:00 +0100 5428262 http://www.medworm.com/index.php?rid=5428261&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058281%26dopt%3DAbstract Authors: Telio D, Pi D, Zalunardo N, Tucker LB, Chen LY PMID: 22058281 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428261 Tue, 01 Nov 2011 04:00:00 +0100 5428261 http://www.medworm.com/index.php?rid=5428260&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D22058282%26dopt%3DAbstract Authors: Asemissen AM, Giagounidis A PMID: 22058282 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5428260 Tue, 01 Nov 2011 04:00:00 +0100 5428260 http://www.medworm.com/index.php?rid=5383532&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993666%26dopt%3DAbstract Conclusions. CD33 is expressed abundantly on immature CD34+/CD38- stem cells and may serve as a stem cell target in chronic myeloid leukemia. PMID: 21993666 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383532 Tue, 11 Oct 2011 04:00:00 +0100 5383532 http://www.medworm.com/index.php?rid=5383531&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993667%26dopt%3DAbstract Conclusions. Our data indicate that CD69 is significantly correlated with poor clinical and biological prognostic factors and is confirmed to be an independent disease prognosticator supporting its introduction in a routine laboratory assessment and, possibly, in a prognostic scoring system for chronic lymphocytic leukemia, after an adequate standardization process. PMID: 21993667 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383531 Tue, 11 Oct 2011 04:00:00 +0100 5383531 http://www.medworm.com/index.php?rid=5383530&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993668%26dopt%3DAbstract Authors: Fried I, Bodner C, Pichler MM, Lind K, Beham-Schmid C, Quehenberger F, Sperr WR, Linkesch W, Sill H, Wolfler A Abstract The recent identification of DNMT3A mutations in de novo acute myeloid leukemia prompted us to determine their frequency, patterns and clinical impact in a cohort of 98 patients with either therapy-related or secondary acute myeloid leukemia developing from an antecedent hematological disorder. We identified 24 somatic mutations in 23 patients with a significantly higher frequency in secondary acute myeloid leukemia (35.1%) as compared to therapy-related acute myeloid leukemia (16.4%, p=0.0486). DNMT3A mutations were associated with a normal karyotype and IDH1/2 mutations, but did not affect survival. In contrast to de novo acute myeloid leukemia, most mu... Haematologica journals http://www.medworm.com/rss/comments.php?id=5383530 Tue, 11 Oct 2011 04:00:00 +0100 5383530 http://www.medworm.com/index.php?rid=5383529&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993669%26dopt%3DAbstract Conclusions. The patient is unique as he displayed features of maturation or shaping of the anti-ADAMTS13 autoantibody response during the course of multiple episodes of thrombotic thrombocytopenic purpura. Anti-ADAMTS13 autoantibodies may be important in vivo despite normal ADAMTS13 activity in routine assays. Consequently, treatment decisions should not be based solely on activity assay results. PMID: 21993669 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383529 Tue, 11 Oct 2011 04:00:00 +0100 5383529 http://www.medworm.com/index.php?rid=5383528&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993670%26dopt%3DAbstract Conclusions. In chronic lymphocytic leukemia, ATM alterations are present at diagnosis in about 25% of individuals, are associated with a peculiar gene expression pattern and a reduced treatment-free interval. PMID: 21993670 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383528 Tue, 11 Oct 2011 04:00:00 +0100 5383528 http://www.medworm.com/index.php?rid=5383527&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993671%26dopt%3DAbstract Conclusions. Children and adults with Chuvash polycythemia have higher estimated right ventricular systolic pressure, even after adjustment for echocardiography estimates of blood volume. Lower ferritin concentration, which is associated with phlebotomy, independently predicts higher tricuspid regurgitation velocity. PMID: 21993671 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383527 Tue, 11 Oct 2011 04:00:00 +0100 5383527 http://www.medworm.com/index.php?rid=5383526&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993672%26dopt%3DAbstract Authors: Maijenburg MW, Kleijer M, Vermeul K, Mul E, van Alphen F, van der Schoot CE, Voermans C Abstract Life-long hematopoiesis depends on the support by mesenchymal stromal cells within the bone marrow. Therefore, changes in the hematopoietic compartment that occur during development and aging probably correlate with variation in the composition of the stromal cell microenvironment. Mesenchymal stromal cells are a heterogeneous cell population and various subtypes may have different functions. In accordance with others, we show that CD271 and CD146 define distinct colony-forming-unit-fibroblast containing mesenchymal stromal cell subpopulations. In addition, analysis of 86 bone marrow samples revealed that the distribution of CD271brightCD146- and CD271brightCD146+ subsets corre... Haematologica journals http://www.medworm.com/rss/comments.php?id=5383526 Tue, 11 Oct 2011 04:00:00 +0100 5383526 http://www.medworm.com/index.php?rid=5383525&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993673%26dopt%3DAbstract Conclusions: The combination of all-trans-retinoic acid and just 2 cycles of idarubicin followed by triple maintenance produced durable remissions in most patients, but patients with high risk disease, especially those with FLT3 mutations, require additional agents or alternative treatment approaches. The significant reduction in relapse seen after the addition of maintenance to the protocol supports a role for maintenance in the context of relatively low chemotherapy exposure during consolidation. (actr.org.au identifier: ACTRN12607000410459). PMID: 21993673 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383525 Tue, 11 Oct 2011 04:00:00 +0100 5383525 http://www.medworm.com/index.php?rid=5383524&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993674%26dopt%3DAbstract Conclusions. Allogeneic stem cell transplantation can result in long-term progression free survival in heavily pre-treated patients with Hodgkin's lymphoma. This reduced intensity conditioning approach has been able to significantly reduce non-relapse mortality; the high relapse rate represents the major remaining challenge in this setting. PMID: 21993674 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383524 Tue, 11 Oct 2011 04:00:00 +0100 5383524 http://www.medworm.com/index.php?rid=5383523&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993675%26dopt%3DAbstract Conclusions. Using a propensity score approach, we found no significant difference in acute myeloid leukemia progression and survival from diagnosis between the cohort treated with lenalidomide and the control cohort. PMID: 21993675 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383523 Tue, 11 Oct 2011 04:00:00 +0100 5383523 http://www.medworm.com/index.php?rid=5383522&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993676%26dopt%3DAbstract Authors: Livadiotti S, Milano GM, Serra A, Folgori L, Jenkner A, Castagnola E, Cesaro S, Rossi MR, Barone A, Zanazzo G, Nesi F, Licciardello M, De Santis R, Ziino O, Cellini M, Porta F, Caselli D, Pontrelli G Abstract A nationwide questionnaire-based survey was designed to evaluate the management and prophylaxis of febrile neutropenia in paediatric patients admitted to Hematology-Oncology and Hematopoietic Stem Cell Transplant Units. Of the 34 participating centres, 40 and 63% respectively, continue to prescribe antibacterial and antimicotic prophylaxis in Low Risk subjects and 78 and 94% in transplant patients. Approximately half of the centers prescribes a combination antibiotic regimen as first-line therapy in Low Risk patients and up to 81% in High Risk patients. When initial e... Haematologica journals http://www.medworm.com/rss/comments.php?id=5383522 Tue, 11 Oct 2011 04:00:00 +0100 5383522 http://www.medworm.com/index.php?rid=5383521&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993677%26dopt%3DAbstract Authors: Day TG, Drasar ER, Fulford T, Sharpe CC, Thein SL Abstract Studies have questioned whether renal dysfunction in sickle cell disease is linked to haemolysis-associated vasculopathy. We have investigated renal function and markers of haemolysis in a cohort of 424 adult African-British patients with sickle cell disease. While significant associations were found in HbSS and HbSβ0 (sickle cell anaemia) patients with and without controlling for covariates between haemolytic markers and albuminuria, the associations were not significant in patients with HbSC. Estimated glomerular filtration rate, a marker of renal function, correlated significantly with reticulocyte count and bilirubin. Alpha thalassaemia, present in 34% of the sickle cell anaemia patients, had a protective effe... Haematologica journals http://www.medworm.com/rss/comments.php?id=5383521 Tue, 11 Oct 2011 04:00:00 +0100 5383521 http://www.medworm.com/index.php?rid=5383520&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993678%26dopt%3DAbstract Conclusions: The Unfolded Protein Response may be a relevant target pathway for proteasome inhibitors in the treatment of myeloma and its regulator XBP-1 is a potential response marker.The BIR study was Australian Clinical Trial Registry Number 12605000770662. PMID: 21993678 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383520 Tue, 11 Oct 2011 04:00:00 +0100 5383520 http://www.medworm.com/index.php?rid=5383519&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993679%26dopt%3DAbstract This study reports the incidence and clinical features of acute promyelocytic leukemia patients treated at Stanford Hospital since March 1997, focusing on early mortality. We show that the risk of early death in acute promyelocytic leukemia patients is higher than previously reported. In a 70 patient cohort that received induction therapy at Stanford Hospital, 19% and 26% died within seven and 30 days of admission, respectively. High early mortality was not limited to our institution as evaluation of the Surveillance, Epidemiology and End Results Database demonstrated that 30-day mortality for acute promyelocytic leukemia averaged 20% from 1977-2007 and did not improve significantly over this interval. Our findings show that early death is now the greatest contributor to treatment failure ... Haematologica journals http://www.medworm.com/rss/comments.php?id=5383519 Tue, 11 Oct 2011 04:00:00 +0100 5383519 http://www.medworm.com/index.php?rid=5383518&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993680%26dopt%3DAbstract ConclusionsHere we report for the first time the influence of position 156 on human leukocyte anti- gen/tapasin association. Additionally, the results of peptide sequencing suggest that tapasin- chaperoning is needed to acquire peptides of unusual length. PMID: 21993680 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383518 Tue, 11 Oct 2011 04:00:00 +0100 5383518 http://www.medworm.com/index.php?rid=5383517&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993681%26dopt%3DAbstract Authors: Bartnikas TB, Fleming MD Abstract Here we investigate the regulation of hepcidin, a hormone that inhibits dietary iron absorption and macrophage iron recycling, by the serum iron-binding protein transferrin. Mice deficient in transferrin (Tfhpx/hpx) and hemojuvelin (Hjv-/-), a gene mutated in juvenile hemochromatosis, a disease of hepcidin deficiency and iron overload, were generated. While Tfhpx/hpx Hjv+/+ and Tfhpx/hpx Hjv-/- phenotypes did not differ markedly, transferrin treatment and RBC transfusions robustly increased hepcidin levels in Tfhpx/hpx Hjv+/+ but not Tfhpx/hpx Hjv-/- mice. These results suggest that, while hemojuvelin is not essential for the establishment or maintenance of hepcidin deficiency in transferrin-deficient mice, hemojuvelin is essential for tra... Haematologica journals http://www.medworm.com/rss/comments.php?id=5383517 Tue, 11 Oct 2011 04:00:00 +0100 5383517 http://www.medworm.com/index.php?rid=5383516&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993682%26dopt%3DAbstract Conclusions. Biochemical and structural parameter did not display significant alterations within the first two weeks of storage, while they constantly declined from day14 onwards.Besides, we highlight several parallelisms between long-stored erythrocytes and hereditary spherocytosis. PMID: 21993682 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383516 Tue, 11 Oct 2011 04:00:00 +0100 5383516 http://www.medworm.com/index.php?rid=5383515&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993683%26dopt%3DAbstract Conclusions. Absolute lymphocyte count/absolute monocyte count ratio at diagnosis is an independent prognostic factor for survival and provides a single biomarker to predict clinical outcomes in classical Hodgkin's lymphoma. PMID: 21993683 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383515 Tue, 11 Oct 2011 04:00:00 +0100 5383515 http://www.medworm.com/index.php?rid=5383514&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993684%26dopt%3DAbstract We report two transfusion-naive siblings with thalassemia intermedia (TI) who clinically deteriorated and became transfusion-dependent shortly following splenectomy. The underlying genotype was coinheritance of IVS1-110 G>A β+ mutation with a complete duplication of the α globin gene cluster on both alleles, leading to 8 functional α globin genes. The father was a carrier of IVS1-110 and has 6 α genes due to α cluster duplication on one allele and shows a moderate-severe TI phenotype. The two children were splenectomized for splenomegaly and associated anemia. Splenectomy in such patients with an absolute excess of α chains may augment peripheral hemolysis leading to worsening anemia. The persistence of procoagulant nucleated red blood cells in the blood stream following splenecto... Haematologica journals http://www.medworm.com/rss/comments.php?id=5383514 Tue, 11 Oct 2011 04:00:00 +0100 5383514 http://www.medworm.com/index.php?rid=5383513&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993685%26dopt%3DAbstract Conclusions Treatment with monoclonal antibody 216 in combination with vincristine is feasible and well tolerated in patients with relapsed or refractory B-cell acute lymphoblastic leukemia. Binding of monoclonal antibody 216 to leukemic blasts was efficient, and favorable early responses were observed. (clinicaltrials.gov identifier 00313053/00313079). PMID: 21993685 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383513 Tue, 11 Oct 2011 04:00:00 +0100 5383513 http://www.medworm.com/index.php?rid=5383512&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993686%26dopt%3DAbstract Authors: Rasi S, Monti S, Spina V, Foa' R, Gaidano G, Rossi D Abstract Recently, two independent investigations of the chronic lymphocytic leukemia (CLL) coding genome have revealed that activating mutations of the NOTCH1 proto-oncogene occur in ~10% CLL at diagnosis. Here we investigated the occurrence of NOTCH1 mutations in 63 consecutive clinical monoclonal B-cell lymphocytosis (cMBL) characterized by a chronic lymphocytic leukemia (CLL)-phenotype. By DNA Sanger sequencing, NOTCH1 mutations occurred in only 2/63 (3.2%) cMBL. In both cases, mutations were represented by a two bp frameshift deletion (c.7544_7545delCT) that represents the most recurrent (~80%) type of NOTCH1 mutation detectable in CLL. Because NOTCH1 mutations in CLL may be subclonal in a fraction of cases, and con... Haematologica journals http://www.medworm.com/rss/comments.php?id=5383512 Tue, 11 Oct 2011 04:00:00 +0100 5383512 http://www.medworm.com/index.php?rid=5383511&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993687%26dopt%3DAbstract Authors: Vettore S, Tezza F, Malara A, Vianello F, Pecci A, Scandellari R, Floris M, Balduini A, Fabris F Abstract Platelet glycoprotein GPIbα mutations are the basic defect behind Bernard-Soulier syndrome, a rare inherited macrothrombocytopenia characterized by anomalies of the GPIbα, GPIbβ and GPIX subunits of von Willebrand factor receptor. A 32-year-old man was investigated for suspected Bernard-Soulier syndrome. Ristocetin induced agglutination was absent. Flow cytometry and Western blot analysis showed a severe reduction of GPIbα, but sequencing revealed only a biallelic c.386A>G substitution, theoretically leading to a p.Asn110Glu variation. To further clarify the data, megakaryocyte cultures were set. Though the maturation of megakaryocytes was normal, the proplatele... Haematologica journals http://www.medworm.com/rss/comments.php?id=5383511 Tue, 11 Oct 2011 04:00:00 +0100 5383511 http://www.medworm.com/index.php?rid=5383510&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993688%26dopt%3DAbstract Conclusions. Patients developing a therapy-related myeloid neoplasm after radioiodine treatment usually present with biological characteristics similar to that seen in patients with therapy-related myeloid neoplasms following other cytotoxic treatment modalities, associated with a low response rate to induction chemotherapy and poor prognosis. PMID: 21993688 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383510 Tue, 11 Oct 2011 04:00:00 +0100 5383510 http://www.medworm.com/index.php?rid=5383509&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21993689%26dopt%3DAbstract Conclusions This study revealed unexpectedly heterogeneous lengths of copy number mutations underlying human coagulopathy. Single nucleotide polymorphism-array had limitations in detecting copy number mutations involving a single exon or those of genes with homologous sequences such as a pseudogene. PMID: 21993689 [PubMed - as supplied by publisher] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383509 Tue, 11 Oct 2011 04:00:00 +0100 5383509 http://www.medworm.com/index.php?rid=5383537&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21972208%26dopt%3DAbstract Authors: Valent P PMID: 21972208 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383537 Sat, 01 Oct 2011 04:00:00 +0100 5383537 http://www.medworm.com/index.php?rid=5383536&cid=s_29484_19_f&fid=29484&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D21972209%26dopt%3DAbstract Authors: Vannucchi AM, Biamonte F PMID: 21972209 [PubMed - in process] (Source: Haematologica) Haematologica journals http://www.medworm.com/rss/comments.php?id=5383536 Sat, 01 Oct 2011 04:00:00 +0100 5383536