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 'European Journal of Haematology' source. Wed, 08 Feb 2012 17:42:00 +0100 http://www.medworm.com/index.php?rid=5649227&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01749.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5649227 Thu, 02 Feb 2012 05:00:00 +0100 5649227 http://www.medworm.com/index.php?rid=5668094&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01764.x AbstractHeat shock protein 90 (HSP90) binds and stabilizes numerous proteins and kinases essential for myeloma cell survival and proliferation. We and others have recently demonstrated that inhibition of HSP90 by small molecular mass inhibitors induces cell death in multiple myeloma (MM). However, some of the HSP90 inhibitors involved in early clinical trials have shown limited antitumor activity and unfavorable toxicity profiles. Here, we analyzed the effects of the novel, orally bioavailable HSP90 inhibitor NVP‐HSP990 on MM cell proliferation and survival. The inhibitor led to a significant reduction in myeloma cell viability and induced G2 cell cycle arrest, degradation of caspases‐8 and ‐3 and induction of apoptosis. Inhibition of the HSP90 ATPase activity was accompanied by degr... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5668094 Wed, 01 Feb 2012 05:00:00 +0100 5668094 http://www.medworm.com/index.php?rid=5649223&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01762.x AbstractEven though the pathogenesis of myelodysplastic syndromes (MDS) is dominated by an inefficient maturation of hematopoietic precursors, also immune mechanisms seem to play a crucial functional role. In this review we will first describe the clinical and laboratory autoimmune manifestations often detectable in MDS patients. We will then focus on studies addressing the mechanisms of T‐cell activation and their implications in the disease history. The potential impact of specific cell subsets, such as regulatory T‐cells, Th17 cells and natural killer cells, will be also described. We will finally focus on potential therapeutic approaches based on immunomodulation, ranging from more classical immunosuppressive drugs to vaccination and transplantation strategies.© 2012 John Wiley &a... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5649223 Wed, 01 Feb 2012 05:00:00 +0100 5649223 http://www.medworm.com/index.php?rid=5649222&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01763.x Conclusions:  Intermittent G‐CSF is effective in maintaining dose intensity in patients receiving ABVD.© 2012 John Wiley & Sons A/S (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5649222 Wed, 01 Feb 2012 05:00:00 +0100 5649222 http://www.medworm.com/index.php?rid=5649221&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01765.x We present the case of a woman with relapsed multiple myeloma (MM) who received combination lenalidomide and bortezomib treatment for 90 cycles followed by continuous lenalidomide monotherapy and has completed over 100 cycles of treatment to date. The patient was diagnosed with advanced stage, symptomatic MM in 2001. Following a partial response (PR) to dexamethasone in combination with pamidronate and thalidomide, the patient underwent protocol‐directed non‐myeloablative allogeneic bone marrow transplantation from her matched sibling donor the following year. In 2004, the patient relapsed and was enrolled in a phase I, dose escalation trial of lenalidomide plus bortezomib for relapsed and refractory MM. After 8 cycles of study treatment, the patient achieved a minimal response. The pa... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5649221 Wed, 01 Feb 2012 05:00:00 +0100 5649221 http://www.medworm.com/index.php?rid=5620898&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01758.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5620898 Mon, 23 Jan 2012 22:05:17 +0100 5620898 http://www.medworm.com/index.php?rid=5620902&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01751.x Conclusion:  Bone demineralization is moderate in multiple myeloma. ASCT induces a decrease of bone resorption but no changes in bone formation, remaining low despite the decrease of DKK1. Bone mineral loss, evaluated by DXA, is moderate in multiple myeloma. High‐dose chemotherapy followed by ASCT leads to decreased bone resorption but osteoblastic bone formation remains low, in spite of reduced circulating DKK1.© 2012 John Wiley & Sons A/S (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5620902 Mon, 23 Jan 2012 05:00:00 +0100 5620902 http://www.medworm.com/index.php?rid=5620901&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01759.x Conclusions:  Low oxygen tension enhanced the proliferation of UCB‐derived HSC/progenitor cells and maintenance of SRCs than normoxia. These expanded cells are expected to be beneficial in the patients who lack human leukocyte antigen (HLA)‐matched donors.© 2012 John Wiley & Sons A/S (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5620901 Mon, 23 Jan 2012 05:00:00 +0100 5620901 http://www.medworm.com/index.php?rid=5620900&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01760.x Conclusion:  The VWF:RCo–ELISA using monoclonal anti‐rfGPIbα antibody SZ‐151 showed improved sensitivity and reliability in detecting VWF:RCo activity, and its clinical application would facilitate the diagnosis and classification of VWD.© 2012 John Wiley & Sons A/S (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5620900 Mon, 23 Jan 2012 05:00:00 +0100 5620900 http://www.medworm.com/index.php?rid=5620899&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01757.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5620899 Mon, 23 Jan 2012 05:00:00 +0100 5620899 http://www.medworm.com/index.php?rid=5610033&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01754.x AbstractFor hemophilia patients with inhibitors, immune tolerance induction (ITI) may help to restore clinical response to Factor (F) VIII or FIX concentrates. Several ITI regimens and protocols exist; however, despite 30 yr of progressive investigation, the ITI evidence base relies mainly on observational data. Expert opinion, experience, and interpretation of the available evidence are therefore valuable to support clinical decision‐making. At the Sixth Zürich Haemophilia Forum an expert panel considered recent data and consensus to distill key practice points relating to ITI. The panel supported current recommendations that, where feasible, ITI should be offered early to children and adults (ideally ≤5 yr of inhibitor detection) when inhibitor titers are <10 Bethesda Units, and ... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5610033 Fri, 20 Jan 2012 21:59:42 +0100 5610033 http://www.medworm.com/index.php?rid=5598804&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01755.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5598804 Tue, 17 Jan 2012 22:55:17 +0100 5598804 http://www.medworm.com/index.php?rid=5660569&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01756.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5660569 Tue, 17 Jan 2012 05:00:00 +0100 5660569 http://www.medworm.com/index.php?rid=5598805&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01756.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5598805 Tue, 17 Jan 2012 05:00:00 +0100 5598805 http://www.medworm.com/index.php?rid=5583359&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01752.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5583359 Sat, 14 Jan 2012 07:29:16 +0100 5583359 http://www.medworm.com/index.php?rid=5561186&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2012.01750.x Conclusions:  This follow‐up has provided for the first time more extensive and detailed information regarding the practice of prophylactic treatment in a large cohort of adults with severe haemophilia. The present study confirms, that early start of prophylaxis and continuing throughout the lifespan has been successful in virtually eliminating joint bleeds, preserving a close to normal joint status, and keeping patients healthy and able to live normal lives.© 2012 John Wiley & Sons A/S (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5561186 Sun, 01 Jan 2012 05:00:00 +0100 5561186 http://www.medworm.com/index.php?rid=5553040&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01747.x Conclusions:  Patients with APL are susceptible to DIC and subsequent bleeding events. Prompt ATRA administration is crucial in preventing hemorrhagic events. High WBC counts, prolonged PT and APTT are associated with clinical bleeding in our series. PT is the most accurate parameter in predicting bleeding. Based on these findings, supportive care should be directed towards correction of coagulopathy to prevent bleeding complications and fresh frozen plasma appears to be indicated for coagulopathy associated with APL.© 2011 John Wiley & Sons A/S (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5553040 Sat, 31 Dec 2011 20:07:11 +0100 5553040 http://www.medworm.com/index.php?rid=5553044&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01748.x Conclusions:  The compound interaction of a β‐thalassemia defect along with a single functional α‐globin gene is quite rare. Although patients with HbH/β‐thal and simple HbH disease, have comparable levels of Hb, the absence of free β‐globin chains and thus detectable non‐functional HbH means that in HbH/β‐thal the levels of functional Hb are higher, resulting in a better compensated functional anemia. Rare large deletions as the one described here remain undetected by gap‐PCR in routine molecular screening. The introduction of MLPA as a diagnostic screening tool may improve laboratory diagnostics for these defects. The use of NimbleGen fine tiling arrays may give additional information about the precise location of breakpoints.© 2011 John Wiley & Sons A/S (Source... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5553044 Fri, 30 Dec 2011 05:00:00 +0100 5553044 http://www.medworm.com/index.php?rid=5553043&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01749.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5553043 Fri, 30 Dec 2011 05:00:00 +0100 5553043 http://www.medworm.com/index.php?rid=5553042&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01745.x Conclusions:  We demonstrated that the number of circulating pDCs is low in patients with primary and H. pylori‐associated ITP, and that it changes depending on treatment modality. Further investigation is warranted with regard to the role of pDCs in the immunopathogenesis of ITP.© 2011 John Wiley & Sons A/S (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5553042 Fri, 30 Dec 2011 05:00:00 +0100 5553042 http://www.medworm.com/index.php?rid=5553041&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01746.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5553041 Fri, 30 Dec 2011 05:00:00 +0100 5553041 http://www.medworm.com/index.php?rid=5526236&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01744.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5526236 Thu, 22 Dec 2011 10:27:29 +0100 5526236 http://www.medworm.com/index.php?rid=5506086&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01724.x Conclusions: After an RIC‐allo, FN and early SBI occurred mostly in patients with severe mucositis and early‐onset neutropenia, while postengraftment high‐dose steroid therapy for acute GVHD was the major RF. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5506086 Fri, 16 Dec 2011 10:29:30 +0100 5506086 http://www.medworm.com/index.php?rid=5506083&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01743.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5506083 Fri, 16 Dec 2011 10:28:20 +0100 5506083 http://www.medworm.com/index.php?rid=5506085&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01741.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5506085 Thu, 15 Dec 2011 05:00:00 +0100 5506085 http://www.medworm.com/index.php?rid=5506084&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01742.x AbstractProtein C (PC) deficiency is an autosomal dominant inherited disorder associated with spontaneous and recurrent thrombotic events. Factor V Leiden (FVL) increases the risk of thrombosis in PC deficient type‐I families. We have investigated the relationship between PC deficiency genotype and clinical phenotype in a large four‐degree Italian family followed since 1988.Methods: PC activity and antigen levels were quantified; sequencing of PC DNA was performed to identify polymorphism. FVL and factor II (G20210A) polymorphism were screened.Results: PC activity ranged from 5% to 9% and PC antigen levels were 5,3% in two homozygous for PROC missense mutation Arg32Cys; PC activity ranged from 18% to 60% and antigen levels from 21% to 64%, respectively in 11 heterozygous for Arg32Cys; ... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5506084 Thu, 15 Dec 2011 05:00:00 +0100 5506084 http://www.medworm.com/index.php?rid=5458839&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01740.x Conclusion:  PINI index appears to be a useful and easy‐to‐perform marker in routine to determine the prognosis of patients with MM, especially in the elderly population. PINI might represent an alternative to ISS score, especially in elderly patients, in the future. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5458839 Thu, 01 Dec 2011 05:00:00 +0100 5458839 http://www.medworm.com/index.php?rid=5561190&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01738.x Abstractβ‐Catenin plays a dual role as a key effecter in the regulation of adherens junctions as well as a transcriptional co‐activator. Tyrosine phosphorylation of β‐catenin affects the cell adhesion, migration, and gene transcription in many types of human cancer cells, including acute myeloid leukemia cells with FLT3 internal tandem duplication (FLT3/ITD‐AML). Here, we investigated the relationship between three tyrosine residues (Y86, Y142, and Y654) in β‐catenin and oncogenic FLT3/ITD kinase. In the experiments using COS‐7 cells expressing FLT3/ITD and Wt or mutant β‐catenin, FLT3/ITD phosphorylated Y654, and this residue was essential for β‐catenin’s nuclear localization by FLT3/ITD. Promoter‐reporter assays demonstrated that Y654 phosphorylation of β‐cate... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5561190 Tue, 29 Nov 2011 05:00:00 +0100 5561190 http://www.medworm.com/index.php?rid=5561189&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01739.x In conclusion, this is the first description of a series of homozygous cases with the A858D mutation. The E5M flowcytometry test is specific for reduction in the Band 3 membrane protein and was useful in conjunction with a careful morphological examination of peripheral blood smears in our patient cohort. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5561189 Tue, 29 Nov 2011 05:00:00 +0100 5561189 http://www.medworm.com/index.php?rid=5458841&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01738.x Abstractβ‐Catenin plays a dual role as a key effecter in the regulation of adherens junctions as well as a transcriptional coactivator. Tyrosine phosphorylation of β‐catenin affects cell adhesion, migration and gene transcription in many types of human cancer cells, including FLT3/ITD‐AML. Here, we investigated the relationship between three tyrosine residues (Y86, Y142, Y654) in β‐catenin and oncogenic FLT3/ITD kinase. In the experiments using COS‐7 cells expressing FLT3/ITD and Wt or mutant β‐catenin, FLT3/ITD phosphorylated Y654, and this residue was essential for β‐catenin’s nuclear localization by FLT3/ITD. Promoter‐reporter assays demonstrated that Y654 phosphorylation of β‐catenin closely related to TCF transcriptional activity. In vitro kinase assays using... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5458841 Tue, 29 Nov 2011 05:00:00 +0100 5458841 http://www.medworm.com/index.php?rid=5458840&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01739.x In conclusion, this is the first description of a series of homozygous cases with the A858D mutation. The E5M flowcytometry test is specific for reduction in the Band 3 membrame protein and was useful in conjunction with a careful morphological examination of peripheral blood smears in our patient cohort. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5458840 Tue, 29 Nov 2011 05:00:00 +0100 5458840 http://www.medworm.com/index.php?rid=5450093&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01737.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5450093 Mon, 28 Nov 2011 10:33:36 +0100 5450093 http://www.medworm.com/index.php?rid=5432443&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01736.x Conclusions:  The fusion genes combining NUP98 exon 11/12 with PSIP1 exon 8, which have never been detected in other AML/CML cases, may be implicated in the pathogenesis of MDS. Furthermore, RQ‐PCR for NUP98/PSIP1 could be useful to monitor minimal residual disease. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5432443 Tue, 22 Nov 2011 10:25:00 +0100 5432443 http://www.medworm.com/index.php?rid=5409590&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01728.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5409590 Thu, 17 Nov 2011 01:26:35 +0100 5409590 http://www.medworm.com/index.php?rid=5409589&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01706.x Conclusions: Cerebral vasculopathy is common in splenectomized adults with TI. However, large‐vessel disease does not explain the occurrence of silent brain infarction. The combined use of MRA and MRI better identifies splenectomized TI adults with neuroimaging abnormalities. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5409589 Thu, 17 Nov 2011 01:26:30 +0100 5409589 http://www.medworm.com/index.php?rid=5409585&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01735.x AbstractLenalidomide (LEN) is a structural analogue of Thalidomide and is currently considered a promising compound among immunomodulatory drugs (IMiDs). Following the demonstration of its potent anti‐angiogenic, anti‐inflammatory and anti‐neoplastic effects in preclinical models, LEN has emerged as an interesting option for the management of selective hematologic malignancies and may also have a possible role in certain solid tumors as well. It is currently approved in the second‐line therapy of multiple myeloma (MM) as well as in myelodysplastic syndrome characterized by 5q minus abnormalities. LEN has been found to be effective in the treatment of both of these conditions and to possess a manageable toxicity profile. In MM, a number of ongoing clinical trials are defining its ro... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5409585 Thu, 17 Nov 2011 01:25:17 +0100 5409585 http://www.medworm.com/index.php?rid=5409587&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01714.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5409587 Tue, 15 Nov 2011 05:00:00 +0100 5409587 http://www.medworm.com/index.php?rid=5409586&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01734.x Conclusions:  This is the first report to study BIRC6 gene in pediatric ALL. Our results suggested that BIRC6 gene expression could be considered as an adverse risk factor in childhood acute leukemia and, hence, could be used to guide therapeutic regimens. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5409586 Tue, 15 Nov 2011 05:00:00 +0100 5409586 http://www.medworm.com/index.php?rid=5495476&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01730.x Conclusions:  Immune thrombocytopenia is more than a bleeding disorder; cognitive symptoms are common and independently associate with autonomic symptoms but not disease severity. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5495476 Wed, 02 Nov 2011 04:00:00 +0100 5495476 http://www.medworm.com/index.php?rid=5388799&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01730.x Conclusion:  ITP is more than a bleeding disorder; cognitive symptoms are common and independently associate with autonomic symptoms but not disease severity. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5388799 Wed, 02 Nov 2011 04:00:00 +0100 5388799 http://www.medworm.com/index.php?rid=5388798&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01731.x Conclusions:  Thus, high index of either CD68 or 163 (>20%) is significantly correlated with poor prognosis in Korean HL patients. CD163, a specific marker of macrophages, seems to be another prognostic factor for classical HL. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5388798 Wed, 02 Nov 2011 04:00:00 +0100 5388798 http://www.medworm.com/index.php?rid=5401342&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01733.x AbstractDeep‐vein thrombosis (DVT) can have a significant impact on a patient’s life. In particular, the development of post‐thrombotic syndrome as a long‐term complication of DVT can have devastating consequences for the individual and impose a substantial economic burden on healthcare systems. Anticoagulants are the mainstay of DVT treatment; however, the current standard of care, a parenteral anticoagulant followed by a vitamin K antagonist, is associated with complex patient management, often resulting in suboptimal therapy. New, oral anticoagulants have been developed, and the direct thrombin inhibitor dabigatran and the direct Factor Xa inhibitors rivaroxaban and apixaban have completed and/or have ongoing phase III trials in the treatment of venous thromboembolism. These age... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5401342 Tue, 01 Nov 2011 04:00:00 +0100 5401342 http://www.medworm.com/index.php?rid=5388797&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01732.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5388797 Tue, 01 Nov 2011 04:00:00 +0100 5388797 http://www.medworm.com/index.php?rid=5348984&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01729.x AbstractThis multicenter prospective randomized trial compared the efficacy and safety of two doses of thalidomide in patients with relapsed or refractory myeloma. The study was designed to test the non‐inferior efficacy and to confirm the better tolerability of low dose thalidomide as compared to a higher dose. 400 patients were randomly assigned to receive either 100 mg/day, or 400 mg/day of thalidomide. Dexamethasone treatment was added in both arms for patients with stable disease or treatment failure at 12 weeks. The primary endpoint was one‐year overall survival (OS).Thalidomide 100 mg/day was better tolerated than 400 mg/day with less high‐grade somnolence, constipation, nausea/vomiting and peripheral neuropathy (P<0.001, P=0.007, P=0.03 and P=0.007, respectively). In the p... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5348984 Wed, 26 Oct 2011 21:42:13 +0100 5348984 http://www.medworm.com/index.php?rid=5561191&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01729.x AbstractThis multicentre prospective randomised trial compared the efficacy and safety of two doses of thalidomide in patients with relapsed or refractory myeloma. The study was designed to test the non‐inferior efficacy and to confirm the better tolerability of low‐dose thalidomide as compared to a higher dose. Four hundred patients were randomly assigned to receive either 100 or 400 mg/day of thalidomide. Dexamethasone treatment was added in both arms for patients with stable disease or treatment failure at 12 weeks. The primary endpoint was 1‐year overall survival (OS). Thalidomide 100 mg/day was better tolerated than 400 mg/day with less high‐grade somnolence, constipation, nausea/vomiting and peripheral neuropathy (P < 0.001, P = 0.007, P = 0.03 and P ... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5561191 Tue, 25 Oct 2011 04:00:00 +0100 5561191 http://www.medworm.com/index.php?rid=5442117&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01726.x AbstractEXtend and eXjange were prospective, 1‐yr, non‐interventional, observational, multicentre studies that investigated deferasirox, a once‐daily oral iron chelator, in iron‐overloaded chelation‐naïve and prechelated patients with myelodysplastic syndromes (MDS), respectively, treated in the daily‐routine setting of office‐based physicians. No inclusion or exclusion criteria or additional monitoring procedures were applied. Deferasirox was administered as recommended in the European Summary of Product Characteristics. Haematological parameters and adverse events (AEs) were collected at two‐monthly intervals. Data from 123 chelation‐naïve patients with MDS (mean age 70.4 yrs) with median baseline serum ferritin level of 2679 (range 184–16 500) ng/mL, and 44 p... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5442117 Mon, 24 Oct 2011 04:00:00 +0100 5442117 http://www.medworm.com/index.php?rid=5423125&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01727.x AbstractTherapy‐related acute promyelocytic leukemia (t‐APL) is a well‐recognized form of APL for which the underlying etiology has been well characterized. The pathogenesis of de novo (dn‐APL) remains unknown; but epidemiologic studies have consistently identified increased body mass index (BMI), younger age, and ethnicity as possible risk factors. We analyzed demographics, clinical features, and treatment responses in a contemporary series of 64 patients treated with all‐trans‐retinoic acid and anthracycline‐based therapy to assess for differences in these two etiologically distinct patient groups. Compared with patients with t‐APL (n = 11), those with dn‐APL (n = 53) had a greater median BMI (31.33 vs. 28.48), incidence of obesity (60.4% vs. 27.3%) (P = 0.0... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5423125 Mon, 24 Oct 2011 04:00:00 +0100 5423125 http://www.medworm.com/index.php?rid=5348988&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01725.x AbstractBarth syndrome (BTHS), a rare, X‐linked, recessive disease characterized by neutropenia and cardiomyopathy. BTHS is caused by loss‐of‐function mutations of the tafazzin (TAZ) gene. We developed a model of BTHS by transfecting human HL60 myeloid progenitor cells with TAZ‐specific shRNAs. Results demonstrate a significant down‐regulation in TAZ expression, mimicking the effects of naturally‐occurring truncation mutations in TAZ. Flow cytometry analyses of cells with TAZ‐specific, but not scrambled, shRNAs demonstrate nearly two‐fold increase in proportion of annexin‐V positive cells and significantly increased dissipation of mitochondrial membrane potential as determined by DIOC6‐staining. Transfection of TAZ specific shRNA had similar effects in U937 myeloid cell... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5348988 Mon, 24 Oct 2011 04:00:00 +0100 5348988 http://www.medworm.com/index.php?rid=5348987&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01724.x Conclusions:  After an RIC‐allo, FN and early SBI occur mostly in patients with severe mucositis and early‐onset neutropenia, while post‐engraftment high‐dose steroid therapy for aGVHD was the major risk factor. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5348987 Mon, 24 Oct 2011 04:00:00 +0100 5348987 http://www.medworm.com/index.php?rid=5348986&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01726.x AbstractEXtend and eXjange were prospective, 1‐year, non‐interventional, observational, multicentre studies that investigated deferasirox, a once‐daily oral iron chelator, in iron‐overloaded chelation‐naïve and pre‐chelated patients with myelodysplastic syndromes (MDS), respectively; treated in the daily‐routine setting of office‐based physicians. No inclusion or exclusion criteria or additional monitoring procedures were applied. Deferasirox was administered as recommended in the European SPC. Haematological parameters and adverse events (AEs) were collected at 2‐monthly intervals. Data from 123 chelation‐naïve MDS patients (mean age 70.4 years) with median baseline serum ferritin level of 2679 (range 184–16,500) ng/mL, and 44 pre‐chelated MDS patients (mean age ... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5348986 Mon, 24 Oct 2011 04:00:00 +0100 5348986 http://www.medworm.com/index.php?rid=5348985&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01727.x Conclusions: Our observations provide further support for the hypothesis that abnormalities in lipid homeostasis may in some way be of pathogenic importance in dn‐APL. Therapy‐related APL is sensitive to standard therapy with no cases of resistance or relapse seen. The inferior OS of the t‐APL was due to induction mortality, possibly reflecting prior therapy. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5348985 Mon, 24 Oct 2011 04:00:00 +0100 5348985 http://www.medworm.com/index.php?rid=5316312&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01723.x AbstractA new mutation (Ile 436 Lys) was found in a cluster of patients in Northeastern Italy. The mutation was present in five patients at the homozygote level and in one patient as a compound heterozygote with an already known mutation namely Glu 117 stop.All these patients showed a mild bleeding tendency mainly associated with deliveries or surgery. The first two patients were two sisters and their parents were consanguineous. The third patient was the only homozygote in the family and parents apparently were not consanguineous. The fourth and fifth patients were a brother and a sister and in this case too, parents were not consanguineous. The sixth patient, a compound heterozygote, negated also the existence of consanguinity between his parents. There were also seven heterozygotes amon... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5316312 Sat, 15 Oct 2011 12:17:48 +0100 5316312 http://www.medworm.com/index.php?rid=5423126&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01723.x AbstractA new mutation (Ile 436 Lys) was found in a cluster of patients in northeastern Italy. The mutation was present in five patients at the homozygote level and in one patient as a compound heterozygote with an already known mutation namely Glu 117 stop. All these patients showed a mild bleeding tendency mainly associated with deliveries or surgery. The first two patients were two sisters, and their parents were consanguineous. The third patient was the only homozygote in the family, and parents apparently were not consanguineous. The fourth and fifth patients were a brother and a sister, and in this case too, parents were not consanguineous. The sixth patient, a compound heterozygote, negated also the existence of consanguinity between his parents. There were also seven heterozygotes ... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5423126 Fri, 14 Oct 2011 04:00:00 +0100 5423126 http://www.medworm.com/index.php?rid=5316313&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01722.x Conclusions:  The aberrant T‐cell expansions associated with the pathogenesis of CIN result in increased proliferation/apoptosis and possibly exhaustion of PB T‐cells which, in association with the inadequate compensatory thymic export of new TREC expressing T‐cells partially due to IL‐7 deficiency, may contribute to lymphopenia in CIN. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5316313 Fri, 14 Oct 2011 04:00:00 +0100 5316313 http://www.medworm.com/index.php?rid=5423127&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01719.x AbstractPlerixafor, a CXCR4 antagonist, has shown to be effective in increasing the number of circulating stem cells, even in patients failing a previous mobilisation attempt. Recently a number of non‐glycosylated recombinant human granulocyte‐colony stimulating factor (G‐CSF) has been clinically approved for the same indications as the originator G‐CSF for comparable safety and efficacy and their reduced cost. In an attempt to provide a less toxic strategy, 14 patients affected by haematological malignancies (non‐Hodgkin’s lymphoma = 4, Hodgkin’s disease = 2 and multiple myeloma = 8), received the combination of biosimilar filgrastim and plerixafor as a first line mobilising strategy. The median number of circulating CD34+ cells on day 4 was 16 (3–42); Plerixaf... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5423127 Wed, 12 Oct 2011 04:00:00 +0100 5423127 http://www.medworm.com/index.php?rid=5316316&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01719.x AbstractPlerixafor, a CXCR4 antagonist, has shown to be effective in increasing the number of circulating stem cells, even in patients failing a previous mobilization attempt. Recently a number of nonglycosylated recombinant human G‐CSF have been clinically approved for the same indications as the originator G‐CSF for comparable safety and efficacy and their reduced cost. In an attempt to provide a less toxic strategy, 14 patients affected by haematological malignancies (Non‐Hodgkin’s Lymphoma=4, Hodgkin’s Disease=2 and Multiple Myeloma=8), received the combination of biosimilar filgrastim and plerixafor as a first line mobilizing strategy. The median number of circulating CD34+ cells on day 4 was 16 (3‐42); Plerixafor was administered to all but 1 patient who had already 42 CD... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5316316 Wed, 12 Oct 2011 04:00:00 +0100 5316316 http://www.medworm.com/index.php?rid=5316315&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01720.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5316315 Wed, 12 Oct 2011 04:00:00 +0100 5316315 http://www.medworm.com/index.php?rid=5316314&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01721.x AbstractOsteolytic bone disease is a hallmark of symptomatic multiple myeloma. Bisphosphonates have been the mainstay of treatment to preserve skeletal integrity and prevent skeletal‐related events in patients with myeloma‐related bone disease. Recently, the MRC Myeloma IX trial demonstrated for the first time improved survival and delayed disease progression with the use of an intravenous amino‐bisphosphonate, zoledronic acid, vs. an oral agent, clodronate, with intensive and non‐intensive anti‐myeloma treatment regimens in patients with newly diagnosed, multiple myeloma. These results validate a large body of preclinical, translational, and other clinical data suggesting anti‐myeloma effects of amino‐bisphosphonates. In addition, this trial also provided the first head‐to... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5316314 Wed, 12 Oct 2011 04:00:00 +0100 5316314 http://www.medworm.com/index.php?rid=5432444&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01718.x Conclusions: In a significant number of patients with chronic ITP, platelet autoantibodies are capable of activating the classical complement pathway. CF is even present in ITP sera without detectable autoantibodies, indicating that current techniques for autoantibody detection may be insufficient. The major targets for complement‐fixing autoantibodies in ITP are GP IIb/IIIa and GP Ib/IX. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5432444 Mon, 10 Oct 2011 04:00:00 +0100 5432444 http://www.medworm.com/index.php?rid=5316317&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01718.x Conclusions:  In a significant number of patients with chronic ITP, platelet autoantibodies are capable of activating the classical complement pathway. Complement fixation is even present in ITP sera without detectable autoantibodies, indicating that current techniques for autoantibody detection may be insufficient. The major targets for complement‐fixing autoantibodies in ITP are GP IIb/IIIa and GP Ib/IX. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5316317 Mon, 10 Oct 2011 04:00:00 +0100 5316317 http://www.medworm.com/index.php?rid=5442118&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01715.x AbstractMyelodysplastic syndromes (MDS) are characterized by dyserythropoiesis resulting in anemia. This pathological hallmark is incompletely understood. Notch signaling has been linked to impaired erythropoietic and megakaryopoietic development of CD34+ progenitor cells, but its role in MDS is unclear. We have analyzed the transcriptional activity of Notch pathway elements and its association with the key erythroid factor globin transcription factor 1 (GATA1) and the apoptosis regulatory gene B‐cell lymphoma‐xl (BCLxl) in MDS. The methylation of GATA1 erythroid promoter CpG dinucleotides flanking cis‐regulatory elements, including an N‐box suppressor binding site for HES1 and a GATA‐box binding site, was examined in normal and MDS erythropoiesis. We have generated a kinetic in ... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5442118 Tue, 04 Oct 2011 04:00:00 +0100 5442118 http://www.medworm.com/index.php?rid=5286653&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01715.x Abstract Myelodysplastic syndromes (MDS) are characterized by dyserythropoiesis resulting in anemia. This pathological hallmark is incompletely understood. Notch signaling has been linked to impaired erythropoietic and megakaryopoietic development of CD34+ progenitor cells; but its role in MDS is unclear. We have analyzed the transcriptional activity of Notch pathway elements and its association with the key erythroid factor GATA1 and the apoptosis regulatory protein BCLxl in MDS. The methylation of GATA1 erythroid promoter CpG dinucleotides flanking cis‐regulatory elements, including an N‐box suppressor binding site for HES1 and a GATA‐box binding site was examined in normal and MDS erythropoiesis. We have generated a kinetic in‐vitro model of MDS erythropoiesis using CD34+ bone m... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5286653 Tue, 04 Oct 2011 04:00:00 +0100 5286653 http://www.medworm.com/index.php?rid=5286652&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01716.x Conclusions:  We have confirmed the high incidence of DNMT3A mutations in patients with AML with IR cytogenetics. Patients with DNMT3A mutations relapse more often and have inferior OS when only patients achieving CR are analyzed. “Double mutated” patients have a very poor prognosis. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5286652 Tue, 04 Oct 2011 04:00:00 +0100 5286652 http://www.medworm.com/index.php?rid=5274787&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01685.x AbstractFor prevention of graft‐versus‐host disease (GVHD), treatment of 24 haematopoietic stem cell transplantation (HSCT) patients with sirolimus and tacrolimus was compared to treatment of matched controls with cyclosporine‐based regimens. The patients mainly had non‐malignant disorders. Two‐thirds of the donors were unrelated, and bone marrow was the most common source of stem cells. Rejection occurred in four patients in the sirolimus group and three in the control group. Donor chimerism for CD3, CD19 and CD33 was similar in the two groups. The cumulative incidence of grade II acute GVHD was 22% in the sirolimus patients and 17% in the controls (P = 0.78). No patients developed acute GVHD of grades III–IV. The cumulative incidence of chronic GVHD was 25% and 37% in the... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5274787 Sun, 02 Oct 2011 04:00:00 +0100 5274787 http://www.medworm.com/index.php?rid=5286651&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01717.x Conclusions:  Our study demonstrates the prognostic impact of eGFRMDRD in MM patients and CKD 5 as the ISS‐independent surrogate predictor of poorest prognosis. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5286651 Sat, 01 Oct 2011 04:00:00 +0100 5286651 http://www.medworm.com/index.php?rid=5261783&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01713.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5261783 Thu, 29 Sep 2011 09:58:22 +0100 5261783 http://www.medworm.com/index.php?rid=5423128&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01713.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5423128 Thu, 29 Sep 2011 04:00:00 +0100 5423128 http://www.medworm.com/index.php?rid=5423124&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01710.x Conclusions:  The results show that trisomy 21‐positive myeloid malignancies are clinically highly variable and that they display a heterogeneous pattern of copy number alterations and mutations. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5423124 Tue, 20 Sep 2011 04:00:00 +0100 5423124 http://www.medworm.com/index.php?rid=5239029&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01709.x AbstractPre‐engraftment syndrome (PES) after umbilical cord blood transplantation (CBT) remains poorly characterized, and the prognosis and appropriate management are unclear. Therefore, we retrospectively analyzed the incidence, risk factors, manifestations, and clinical outcomes of PES in CBT recipients, who had been treated for hematological malignancies at our transplantation center. PES was defined as unexplained fever higher than 38.3°C that is not associated with documented infection and unresponsive to antimicrobial manipulations; and/or unexplained erythematous skin rash occurring prior to neutrophil engraftment. A total of 81 patients (median 18 years, range 3‐48) received either myeloablative (n=72) or nonmyeloablative (n=9) conditioning. Neutrophil engraftment was achieved... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5239029 Tue, 20 Sep 2011 04:00:00 +0100 5239029 http://www.medworm.com/index.php?rid=5239028&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01710.x Conclusions:  The results show that trisomy 21‐positive myeloid malignancies are clinically highly variable and that they display a heterogeneous pattern of copy number alterations and mutations. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5239028 Tue, 20 Sep 2011 04:00:00 +0100 5239028 http://www.medworm.com/index.php?rid=5409588&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01708.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5409588 Thu, 15 Sep 2011 04:00:00 +0100 5409588 http://www.medworm.com/index.php?rid=5227155&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01706.x Conclusions:  Cerebral vasculopathy is common in splenectomized adults with TI. However, large‐vessel disease does not explain the occurrence of silent brain infarction. The combined use of MRA and MRI better identifies splenectomized TI adults with neuroimaging abnormalities. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5227155 Tue, 13 Sep 2011 04:00:00 +0100 5227155 http://www.medworm.com/index.php?rid=5227154&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01707.x Conclusions:  Our results suggest essential roles of specific microRNAs in regulating immune function of CB cells, providing insight into the underlying molecular mechanism. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5227154 Tue, 13 Sep 2011 04:00:00 +0100 5227154 http://www.medworm.com/index.php?rid=5251501&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01712.x AbstractWe investigated the role of lipocalin‐2 (LCN‐2) and its receptor (SLC22A17) in mediating clonal dominance in a patient with both BCR‐ABL and JAK2‐V617F mutations. LCN‐2 mRNA showed a near 50‐fold increase in expression, accompanied by downregulation of SLC22A17, coinciding with increase in BCR‐ABL transcripts, loss of JAK2‐V617F and change of clinical phenotype from polycythaemia vera to chronic myeloid leukaemia. These changes were reversed after commencing imatinib mesylate. Consistent with experimental studies, BCR‐ABL+ cells express LCN‐2 leading to suppression of BCR‐ABL‐ cells and explain their eventual dominance when occurring together with JAK2‐V617F. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5251501 Thu, 01 Sep 2011 04:00:00 +0100 5251501 http://www.medworm.com/index.php?rid=5239027&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01711.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5239027 Thu, 01 Sep 2011 04:00:00 +0100 5239027 http://www.medworm.com/index.php?rid=5227153&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01708.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5227153 Thu, 01 Sep 2011 04:00:00 +0100 5227153 http://www.medworm.com/index.php?rid=5210092&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01705.x AbstractIn order to validate the recently proposed pediatric severity score (PSS) for sickle cell disease (SCD), we retrospectively assembled clinical data from a cohort of 122 SCD patients (105 S/S or S/β0‐thal. and 17 S/C) followed for at least two years. Besides age and α‐ and β‐globin genotypes, four new parameters were also tested against the PSS: duration of data assembly, neonatal screening, use of transcranial Doppler ultrasound to prevent vasculopathies and β‐globin gene cluster haplotype.Once again, the PSS clearly differentiated patients by their β‐globin genotype (p = 0.004) but not by their age during data assembly (p = 0.159). But, surprisingly, alpha‐gene deletions were not associated with a lower PSS (p = 0.604), possibly reflecting the opposite effects of ... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5210092 Thu, 01 Sep 2011 04:00:00 +0100 5210092 http://www.medworm.com/index.php?rid=5184900&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01703.x AbstractThe combination of cytoreductive chemotherapy with reduced intensity conditioning (RIC) is a highly‐effective anti‐leukemic therapy. Purpose of this retrospective analysis was to evaluate the anti‐leukemic efficacy and toxicity of clofarabine‐based chemotherapy followed by RIC and allogeneic stem cell transplantation (SCT) for high‐risk, relapsed or refractory acute myeloid leukemia or myelodysplastic syndromes (MDS). From May 2007 until October 2009 a total of 27 patients underwent allogeneic SCT after treatment with clofarabine and ara‐C for five days and RIC (4 Gy TBI/cyclophosphamide/ATG). Prophylaxis of graft versus host‐disease (GvHD) consisted of cyclosporine and mycophenolate mofetil. Unmanipulated G‐CSF mobilized PBSC (n = 26) or bone marrow cells (n = 1) w... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5184900 Wed, 31 Aug 2011 23:00:00 +0100 5184900 http://www.medworm.com/index.php?rid=5423129&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01703.x AbstractThe combination of cytoreductive chemotherapy with reduced‐intensity conditioning (RIC) is a highly effective antileukemic therapy. Purpose of this retrospective analysis was to evaluate the antileukemic efficacy and toxicity of clofarabine‐based chemotherapy followed by RIC and allogeneic stem cell transplantation (SCT) for high‐risk, relapsed, or refractory acute myeloid leukemia (AML) or myelodysplastic syndromes (MDS). From May 2007 until October 2009, a total of 27 patients underwent allogeneic SCT after treatment with clofarabine and ara‐C for 5 d and RIC (4 Gy TBI/cyclophosphamide/ATG). Prophylaxis of graft‐versus‐host disease (GvHD) consisted of cyclosporine and mycophenolate mofetil. Unmanipulated G‐CSF mobilized PBSC (n = 26) or bone marrow cells (n�... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5423129 Wed, 31 Aug 2011 04:00:00 +0100 5423129 http://www.medworm.com/index.php?rid=5184903&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01700.x AbstractFew risk factors have been established for the plasma cell disorder multiple myeloma, but some of these like African American ethnicity and a family history of B‐cell lymphoproliferative diseases suggest a genetic component for the disease. Genetic variation represents the genetic basis of variability in a population. The complex interplay between environment and genes for development of cancer may therefore be influenced by genetic variations. A genetic variation may change the function of the gene and if the genetic variation is associated with risk of disease, that particular gene may be involved in the pathogenesis of disease. Genes of interest are genes involved in the normal development and function of the plasma cell and genes that protect us against exposures from the env... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5184903 Tue, 30 Aug 2011 23:00:00 +0100 5184903 http://www.medworm.com/index.php?rid=5184902&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01701.x AbstractParoxysmal nocturnal hemoglobinuria (PNH) is a rare, life‐threatening and debilitating clonal blood disorder caused by an acquired mutation in the phosphatidylinositol glycan (PIG)‐A gene. In pluripotent hematopoetic stem cells, this leads to a deficiency of glycosylphosphatidylinositol (GPI)‐anchors and GPI‐anchored proteins, including the complement regulators CD55 and CD59, on the surface of affected blood cells. PNH red blood cells are highly vulnerable to activation of complement and the formation of the membrane attack complex (MAC). The resulting chronic intravascular hemolysis is the underlying cause of PNH morbidities and mortality. Until recently, the treatment of PNH has been largely empirical and symptomatic with blood transfusions, anticoagulation, and suppleme... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5184902 Tue, 30 Aug 2011 23:00:00 +0100 5184902 http://www.medworm.com/index.php?rid=5184901&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01702.x Conclusions:  The incidence of GVHD was lower than in other series using conventional myeloablative preparative regimens. Most important, the severity of GVHD did not significantly affect long‐term survival. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5184901 Tue, 30 Aug 2011 23:00:00 +0100 5184901 http://www.medworm.com/index.php?rid=5170923&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01704.x Conclusion:  Analysis of real‐life data from one single centre in Germany demonstrated a change in antifungal management of AML patients between 2004/5 and 2006, accompanied by a decline in total costs. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5170923 Mon, 29 Aug 2011 15:49:36 +0100 5170923 http://www.medworm.com/index.php?rid=5316318&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01704.x Conclusion: Analysis of real‐life data from one single centre in Germany demonstrated a change in antifungal management of patients with AML between 2004/2005 and 2006, accompanied by a decline in total costs. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5316318 Mon, 29 Aug 2011 04:00:00 +0100 5316318 http://www.medworm.com/index.php?rid=5274785&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01697.x Conclusion: This study shows that RIC improved OS by directly lowering TRM and indirectly giving an additional chance for relapsed MDS in the era of hypomethylating treatment. RIC–SCT should be considered for relative healthy lower‐risk MDS patients. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5274785 Sun, 28 Aug 2011 04:00:00 +0100 5274785 http://www.medworm.com/index.php?rid=5274784&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01698.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5274784 Sun, 28 Aug 2011 04:00:00 +0100 5274784 http://www.medworm.com/index.php?rid=5274783&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01699.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5274783 Sun, 28 Aug 2011 04:00:00 +0100 5274783 http://www.medworm.com/index.php?rid=5170927&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01696.x AbstractAssociation studies on genetic variation to treatment effect may serve as a predictive marker for effect of treatment but can also uncover biological pathways behind drug effect. Single nucleotide polymorphisms (SNPs) have been studied in relation to high‐dose treatment (HDT), thalidomide and bortezomib based therapy, maintenance treatment with interferon‐α and in relation to therapy related adverse effects caused by treatment. Candidate genes for prediction of effect of HDT include DNA repair genes, CYP genes and genes involved in inflammation and apoptosis such as IL1B and RAI. In thalidomide and bortezomid based therapy candidate genes include TNFA and genes involved in the NF‐κB pathway (NFKB2 and TRAF3), respectively. In maintenance treatment with interferon‐α, a po... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5170927 Sat, 27 Aug 2011 23:00:00 +0100 5170927 http://www.medworm.com/index.php?rid=5170926&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01697.x Conclusion:  This study shows that RIC improved OS by directly lowering TRM and indirectly giving an additional chance for relapsed MDS in the era of hypomethylating treatment. RIC‐SCT should be considered for relative healthy lower‐risk MDS patients. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5170926 Sat, 27 Aug 2011 23:00:00 +0100 5170926 http://www.medworm.com/index.php?rid=5170925&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01698.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5170925 Sat, 27 Aug 2011 23:00:00 +0100 5170925 http://www.medworm.com/index.php?rid=5170924&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01699.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5170924 Sat, 27 Aug 2011 23:00:00 +0100 5170924 http://www.medworm.com/index.php?rid=5144895&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01663.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5144895 Sat, 20 Aug 2011 17:00:19 +0100 5144895 http://www.medworm.com/index.php?rid=5144894&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01675.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5144894 Sat, 20 Aug 2011 17:00:17 +0100 5144894 http://www.medworm.com/index.php?rid=5144884&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01695.x Abstract:Trimethoprim‐sulfamethoxazole (TMP/SMX) is used in children with acute lymphoblastic leukaemia to prevent Pneumocystis pneumonia (PCP). We explored to which extent TMP/SMX influenced methotrexate (MTX)/6‐mercaptopurine (6MP) dosage, myelosuppression, and event‐free survival during maintenance therapy. Of 447 study patients treated by the NOPHO ALL92 protocol, 120 patients received TMP/SMX continuously 2‐7 days/week (TMP/SMX2‐7) and 287 patients never received TMP/SMX (TMP/SMXnever). Ten patients (all TMP/SMXnever) developed PCP, 8 of which occurred within 7 months from the start of maintenance therapy. The TMP/SMX2‐7 group received lower oral 6MP doses than TMP/SMXnever patients (50.6 vs. 63.9 mg/m2/day; P<0.001), but had lower absolute neutrophil counts (ANC) (medi... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5144884 Sat, 20 Aug 2011 16:59:35 +0100 5144884 http://www.medworm.com/index.php?rid=5274786&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01695.x AbstractTrimethoprim‐sulfamethoxazole (TMP/SMX) is used in children with acute lymphoblastic leukemia (ALL) to prevent Pneumocystis pneumonia (PCP). We explored to which extent TMP/SMX influenced methotrexate (MTX)/6‐mercaptopurine (6MP) dosage, myelosuppression, and event‐free survival (EFS) during maintenance therapy. Of 447 study patients treated by the NOPHO ALL92 protocol, 120 patients received TMP/SMX continuously for 2–7 d/wk (TMP/SMX2–7) and 287 patients never received TMP/SMX (TMP/SMXnever). Ten patients (all TMP/SMXnever) developed PCP, eight of which occurred within 7 months from the start of maintenance therapy. The TMP/SMX2–7 group received lower oral 6MP doses than TMP/SMXnever patients (50.6 vs. 63.9 mg/m2/d; P < 0.001) but had lower absolute neutrop... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5274786 Sat, 20 Aug 2011 04:00:00 +0100 5274786 http://www.medworm.com/index.php?rid=5239030&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01692.x Conclusion:  Although we failed to demonstrate any advantage of PBSCT over conventional BMT with regard to overall survival, relapse incidence and non‐relapse mortality PBSCT were associated with a significantly higher incidence of chronic graft‐versus‐host disease. Therefore, and by virtue of observations, that some patient groups might benefit from either stem cell source, there is still need for prospective randomized trials with special emphasize on quality of life in long‐term survivors. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5239030 Wed, 17 Aug 2011 04:00:00 +0100 5239030 http://www.medworm.com/index.php?rid=5144888&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01691.x Conclusions:  These findings indicate that ‐765G/C polymorphism in COX‐2 may play a vital role in mediating individual susceptibility to AML. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5144888 Tue, 16 Aug 2011 23:00:00 +0100 5144888 http://www.medworm.com/index.php?rid=5144887&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01692.x Conclusion:  Although we failed to demonstrate any advantage of peripheral blood stem cell transplantation over conventional bone marrow transplantation with regard to overall survival, relapse incidence, and non‐relapse mortality peripheral blood stem cell transplantation was associated with a significantly higher incidence of chronic graft‐versus‐host disease. Therefore, and by virtue of observations, that some patient groups might benefit from either stem cell source, there is still need for prospective randomized trials with special emphasize on quality of life in long‐term survivors. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5144887 Tue, 16 Aug 2011 23:00:00 +0100 5144887 http://www.medworm.com/index.php?rid=5144886&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01693.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5144886 Tue, 16 Aug 2011 23:00:00 +0100 5144886 http://www.medworm.com/index.php?rid=5144885&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01694.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5144885 Tue, 16 Aug 2011 23:00:00 +0100 5144885 http://www.medworm.com/index.php?rid=5098685&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01689.x AbstractA revolution in medical science was marked with the advent of imatinib, a site specific drug for the management of patients with chronic myeloid leukemia (CML). Imatinib mesylate (also known as Glivec, Gleevec, STI‐571, CGP57148), an orally administered 2‐phenylaminopyrimidine derivative approved by FDA in 2001 for treatment of CML is highly effective in treating the early stages of chronic myeloid leukemia, but remission induced in advanced phase were observed to be relatively short lived. The primary cause of resistance in CML patients is the mutations in the BCR/ABL kinase domain. This review discusses the different mechanisms leading to Imatinib resistance and various treatment options to over ride imatinib resistance. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5098685 Wed, 03 Aug 2011 23:00:00 +0100 5098685 http://www.medworm.com/index.php?rid=5120453&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01686.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5120453 Sun, 31 Jul 2011 23:00:00 +0100 5120453 http://www.medworm.com/index.php?rid=5098684&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01690.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5098684 Sun, 31 Jul 2011 23:00:00 +0100 5098684 http://www.medworm.com/index.php?rid=5080764&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01688.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5080764 Sun, 31 Jul 2011 23:00:00 +0100 5080764 http://www.medworm.com/index.php?rid=5227156&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01687.x AbstractA novel β‐globin gene promoter (−71 C>T) nucleotide change was recently posted to the HbVar database (ID 2701) without precision on phenotype and ethnicity. We found the same change in compound heterozygosity with Hb S [β6(A3)Glu>Val] in an Omani family with almost equal expression of Hb A and Hb S. This suggested that the −71 C to T mutation may be a mild β‐thalassemic allele. Subsequent search found three other independent cases with the same atypical Hb A:Hb S ratio, further confirming the mild thalassemic feature of this mutation. In addition, molecular screening of a set of subjects (with only Hb A) with borderline Hb A2 or MCV values revealed the presence of −71 C>T change in heterozygous state, altogether assigning the mutation as a mild β+ thalassemic... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5227156 Fri, 29 Jul 2011 04:00:00 +0100 5227156 http://www.medworm.com/index.php?rid=5080765&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01687.x AbstractA novel β‐globin gene promoter (‐71 C>T) nucleotide change was recently posted to the HbVar database (ID 2701) without precision on phenotype and ethnicity. We found the same change in compound heterozygosity with HbS [β6(A3)Glu>Val] in an Omani family with almost equal expression of HbA and HbS. This suggested that the ‐71 C to T mutation may be a mild β‐thalassemic allele. Subsequent search found three other independent cases with the same atypical HbA:HbS ratio, further confirming the mild thalassemic feature of this mutation. In addition, molecular screening of a set of subjects (with only HbA) with borderline Hb A2 or MCV values revealed the presence of ‐71 C>T change in heterozygous state, altogether assigning the mutation as a mild β+ thalassemic allel... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5080765 Thu, 28 Jul 2011 23:00:00 +0100 5080765 http://www.medworm.com/index.php?rid=5064307&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01630.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5064307 Mon, 25 Jul 2011 23:00:00 +0100 5064307 http://www.medworm.com/index.php?rid=5031271&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01653.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031271 Sat, 16 Jul 2011 17:31:40 +0100 5031271 http://www.medworm.com/index.php?rid=5031270&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01628.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031270 Sat, 16 Jul 2011 17:31:39 +0100 5031270 http://www.medworm.com/index.php?rid=5031269&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01643.x AbstractAlthough maternal haematopoiesis is characterised by rapid proliferation and immunological adjustment, leukaemia seldom occurs in pregnant women. In this case–control study, we investigated pregnancy and risk of acute myeloid leukaemia (AML). A total of 785 women with AML diagnosed in ages 15–50 were compared with 1576 age‐ and sex‐matched controls. At the time of diagnosis, 13 cases and 53 controls were pregnant (1.3% and 3.4%, respectively), resulting in a significantly reduced odds ratio of 0.44 (95% confidence interval 0.22–0.85). Odds ratios of AML during the years following childbirth were close to unity. The results suggest that pregnancy conveys a strong short‐term protection against AML. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031269 Sat, 16 Jul 2011 17:31:37 +0100 5031269 http://www.medworm.com/index.php?rid=5031268&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01637.x Conclusions: The ability to achieve results comparable to those of previous clinical studies in our CML cohort was influenced by centralised care. Decisions not to initiate imatinib or to delay AHSCT may have a negative impact on OS, but comorbidities may limit the treatment potential of imatinib in the elderly. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031268 Sat, 16 Jul 2011 17:31:35 +0100 5031268 http://www.medworm.com/index.php?rid=5031267&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01638.x Conclusions:  A significant proportion of long‐term survivors of HSCT had late complications. cGvHD remained an important risk factor for late complications despite T‐cell depletion resulting in immunosuppression and infectious complications. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031267 Sat, 16 Jul 2011 17:31:33 +0100 5031267 http://www.medworm.com/index.php?rid=5031266&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01639.x AbstractObjectives: The impact of modern prognostic markers on clinical course of chronic lymphocytic leukaemia (CLL) in everyday practice has been not yet well defined, especially in large series of patients. Therefore, the goal of this study was to assess the influence of conventional as well as modern prognostic factors on overall survival (OS) and time to therapy (TTT) of patients with CLL. Methods: We retrospectively analysed data of all patients consecutively entered into the databases of five large academic centres in the Czech Republic. The total of 1300 patients was included in the analysis. Results and conclusion: Through the use of uniparametric analysis, it was determined that gender, clinical stage Rai II–IV, unmutated IgVH status, deletion 17p (for both 5% and 20% cut‐off... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031266 Sat, 16 Jul 2011 17:31:31 +0100 5031266 http://www.medworm.com/index.php?rid=5031265&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01634.x Conclusion:  For patients with a long‐lasting response after ASCT, MEL 100 could be a therapeutic option with low toxicity and with efficacy comparable to newer immunomodulatory drugs. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031265 Sat, 16 Jul 2011 17:31:28 +0100 5031265 http://www.medworm.com/index.php?rid=5031264&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01636.x This report presents a concise review of the pathophysiological mechanisms of AVWD in these various underlying conditions. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031264 Sat, 16 Jul 2011 17:31:26 +0100 5031264 http://www.medworm.com/index.php?rid=5031260&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01684.x Conclusion:  Rearrangement or amplification involving the BCL3 gene, is a rare event in DLBCL but is likely to play a role in the pathogenesis of a minority of de novo DLBCL. BCL3 over‐expression is more frequent and occurs in the absence of rearrangement or amplification, and is a feature of the non‐GC subset of DLBCL. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031260 Sat, 16 Jul 2011 17:30:50 +0100 5031260 http://www.medworm.com/index.php?rid=5144891&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01684.x Conclusion: Rearrangement or amplification involving the BCL3 gene is a rare event in DLBCL but is likely to play a role in the pathogenesis of a minority of de novo DLBCL. BCL3 over‐expression is more frequent and occurs in the absence of rearrangement or amplification and is a feature of the non‐GC subset of DLBCL. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5144891 Wed, 13 Jul 2011 23:00:00 +0100 5144891 http://www.medworm.com/index.php?rid=5227157&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01681.x Conclusion:  SPCD34+ cells in PBPC define an immature phenotype of HPC with increased numbers of LTC‐IC, and they are more frequently found in PBPC than in BM. The number of SP cells does not predict time to engraftment. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5227157 Wed, 13 Jul 2011 04:00:00 +0100 5227157 http://www.medworm.com/index.php?rid=5031263&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01681.x Conclusion:  SPCD34+ cells in PBPC define an immature phenotype of hematopoietic progenitor cells with increased numbers of LTC‐IC, and they are more frequently found in PBPC than in BM. The number of SP cells does not predict time to engraftment. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031263 Tue, 12 Jul 2011 23:00:00 +0100 5031263 http://www.medworm.com/index.php?rid=5031262&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01682.x This article discusses the epidemiology of the most important fungal pathogens, identifies high‐risk patient groups and risk factors associated with IFI, and critically evaluates the advantages and disadvantages of available diagnostic tests and treatment strategies and the rationale for antifungal prophylaxis. For patients at high risk for IFI, antifungal prophylaxis is an attractive strategy and numerous randomized, controlled clinical studies have documented the benefit of such prophylaxis as well as the most efficacious of currently available agents. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031262 Tue, 12 Jul 2011 23:00:00 +0100 5031262 http://www.medworm.com/index.php?rid=5031261&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01683.x AbstractPatients with recurring T‐cell non‐Hodgkin lymphoma (T‐NHL) are incurable and candidate to investigational agents. Here, we report on five patients with T‐NHL refractory to multiple chemotherapy lines, including in all cases alkylators and gemcitabine, who received the 3rd‐generation chloroethylnitrosourea fotemustine at a dose of 120 mg/m2 every 21 days, up to 8 courses. Median actual dose intensity was 79%; toxicity was manageable and mainly haematological. One complete remission, one partial remission, two protracted disease stabilization and one transient, minor response were achieved. Time to progression ranged from 48 to 240+ days. This is the first evidence ever reporting activity of fotemustine in end‐stage T‐NHL. Formal studies with this agent are warranted i... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5031261 Tue, 12 Jul 2011 23:00:00 +0100 5031261 http://www.medworm.com/index.php?rid=5163389&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01680.x AbstractDespite some considerable progress in the therapy for chronic lymphocytic leukaemia (CLL) owing to fludarabine‐based regimens and rituximab, no curative treatment is available so far. We conducted an explorative phase II study in patients with CLL, prolymphocytic leukaemia (PLL) and leukaemic lymphoplasmacytic lymphoma (LL) with the combination of fludarabine, epirubicin and rituximab (FER) to improve the complete remission (CR) rate and progression‐free survival (PFS). Fludarabine 25 mg/m2 was administered i.v. on days 1–5 and epirubicin 25 mg/m2 i.v. on days 4 and 5, and rituximab was added at a dose of 375 mg/m2 i.v. day 1 in the first cycle and at a dose of 500 mg/m2 in all consecutive cycles. Patients exhibiting responsive disease after FER were eligible to recei... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5163389 Mon, 11 Jul 2011 23:00:00 +0100 5163389 http://www.medworm.com/index.php?rid=5021741&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01679.x Conclusions:  Patients with bulky disease had a poorer prognosis and recurred frequently in the CNS. CNS prophylaxis might yield better outcomes, but a larger, prospective trial is needed to elucidate the optimal initial treatment of PBL in the rituximab era. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5021741 Sat, 09 Jul 2011 23:00:00 +0100 5021741 http://www.medworm.com/index.php?rid=5070554&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01678.x Conclusions:  We demonstrate for the first time that patients receiving intensive chemotherapy for acute leukemia have the highest risk of developing IMI during their treatment compared to patients with allogeneic SCT. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5070554 Tue, 05 Jul 2011 23:00:00 +0100 5070554 http://www.medworm.com/index.php?rid=5120454&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01677.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5120454 Thu, 30 Jun 2011 23:00:00 +0100 5120454 http://www.medworm.com/index.php?rid=5021740&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01680.x AbstractDespite some considerable progress in the therapy of CLL due to fludarabine based regimens and rituximab, no curative treatment is available so far. We conducted an explorative phase‐II study in patients with CLL, PLL and leukaemic lymphoplasmacytic lymphoma (LL) with the combination of fludarabine, epirubicin and rituximab (FER) to improve the CR rate and PFS.Fludarabine 25mg/m² was administered i.v. on days 1 to 5, epirubicin 25mg/m² i.v. on days 4 and 5, and rituximab was added at a dose of 375mg/m² i.v. day 1 in the first cycle and at a dose of 500mg/m² in all consecutive cycles. Patients exhibiting responsive disease after FER were eligible to receive maintenance therapy of up to 12 cycles of rituximab 375mg/m² bimonthly.44 patients (38 CLL, 4 PLL, 2 LL) with a median a... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5021740 Thu, 30 Jun 2011 23:00:00 +0100 5021740 http://www.medworm.com/index.php?rid=5000040&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01678.x Conclusions:  We demonstrate for the first time that patients receiving intensive chemotherapy for acute leukaemia have the highest risk of developing IMI during their treatment compared to patients with allogeneic SCT. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5000040 Thu, 30 Jun 2011 23:00:00 +0100 5000040 http://www.medworm.com/index.php?rid=4986943&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01677.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4986943 Thu, 30 Jun 2011 23:00:00 +0100 4986943 http://www.medworm.com/index.php?rid=4978210&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01676.x Conclusions:  Flow cytometric analysis in MDS may provide meaningful prognostic information. Blast percentage expressed as CD117+ or CD34+ cells and the quantitative assessment of myeloid maturation showed prognostic value for survival. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4978210 Wed, 29 Jun 2011 18:04:00 +0100 4978210 http://www.medworm.com/index.php?rid=4978211&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01674.x SummarySeveral pediatric patients showing symptoms consistent with the Wiskott‐Aldrich syndrome (WAS) were referred to us and turned out to display the c.273+11dup change in the WAS gene. It consisted of the insertion of one C in an unusual tract of 7C near the intron 2 donor splicing site of the WAS gene. In the patients, non‐synonymous WAS mutations were found twice only and one mutation was elucidated in RUNX1. In the absence of a non‐synonymous mutation in the WAS gene, the c.273+11dup change affected neither the levels nor the sequence of WAS mRNA. In the presence of a non‐synonymous WAS mutation, the c.273+11dup alteration failed to worsen the expected phenotype. Minor splicing abnormalities concerning exon 10 were observed both in WAS patients, and in healthy individuals car... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4978211 Mon, 27 Jun 2011 23:00:00 +0100 4978211 http://www.medworm.com/index.php?rid=4971158&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01673.x AbstractMutation in the NPM1 gene occurs in 60% of acute myeloid leukaemia (AML) patients with normal karyotype. NPM1 mutation is potentially a superior minimal residual disease (MRD) marker compared to WT1 gene overexpression by being specific to the malignant clone, although experimental evidence published so far includes very limited numbers of relapsed cases. Also, the stability of the NPM1 mutation has been questioned by reports of the mutation being lost at relapse. In the present study we compared NPM1 mutation and WT1 overexpression as MRD markers in 20 cases of relapsed AML. The 20 patients experienced a total of 28 morphological relapses. Karyotypic evolution was detected in 56% of relapses. All relapses were accompanied by high levels of NPM1 mutation, along with high WT1 mRNA l... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4971158 Mon, 27 Jun 2011 19:36:08 +0100 4971158 http://www.medworm.com/index.php?rid=4971160&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01672.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4971160 Thu, 23 Jun 2011 23:00:00 +0100 4971160 http://www.medworm.com/index.php?rid=4971159&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01671.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4971159 Thu, 23 Jun 2011 23:00:00 +0100 4971159 http://www.medworm.com/index.php?rid=4955178&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01625.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955178 Wed, 22 Jun 2011 17:58:03 +0100 4955178 http://www.medworm.com/index.php?rid=4955177&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01624.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955177 Wed, 22 Jun 2011 17:58:03 +0100 4955177 http://www.medworm.com/index.php?rid=4955176&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01621.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955176 Wed, 22 Jun 2011 17:58:02 +0100 4955176 http://www.medworm.com/index.php?rid=4955175&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01613.x Conclusion: We conclude that the chemokine receptor CCR4 is not a special feature of CLL cells with skin manifestation, but rather it is expressed in a low but significant proportion of peripheral blood CLL cells. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955175 Wed, 22 Jun 2011 17:58:01 +0100 4955175 http://www.medworm.com/index.php?rid=4955174&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01623.x Conclusion: Patients with transfusion‐independent TI have lower HR‐QoL compared to TM patients. At a comparable age, the shorter duration since diagnosis and the multiplicity of complications may explain these findings. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955174 Wed, 22 Jun 2011 17:58:00 +0100 4955174 http://www.medworm.com/index.php?rid=4955173&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01616.x Conclusion: Although Hb Nakhon Ratchasima might be clinically innocuous, differential diagnosis from other clinically relevant hemoglobinopathies is essential in routine setting. This could be made by using a simple PCR–restriction fragment length polymorphism assay or allele‐specific PCR assay developed in this study. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955173 Wed, 22 Jun 2011 17:57:59 +0100 4955173 http://www.medworm.com/index.php?rid=4955172&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01620.x Conclusion:  This study revealed that in spite of decrease in estimated pulmonary hypertension in treated group, HU has no statistically significant effect on pulmonary acceleration time and M‐mode, Doppler, and tissue Doppler changes of patients with TI. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955172 Wed, 22 Jun 2011 17:57:58 +0100 4955172 http://www.medworm.com/index.php?rid=4955171&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01618.x AbstractGene expression profiling studies have unraveled deregulation of several genes that might be of pathogenetic importance for the development and phenotype of the Philadelphia‐negative chronic myeloproliferative neoplasms. In the context of interferon‐alpha2 as a promising therapeutic agent, we focused upon the transcriptional profiling of interferon‐associated genes in patients with essential thrombocythemia (ET) (n = 19), polycythemia vera (PV) (n = 41), and primary myelofibrosis (PMF) (n = 9). Using whole‐blood transcriptional profiling and accordingly obtaining an integrated signature of genes expressed in several immune cells (granulocytes, monocytes, B cells, T cells, platelets), we have identified a number of interferon‐associated genes to be significantl... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955171 Wed, 22 Jun 2011 17:57:57 +0100 4955171 http://www.medworm.com/index.php?rid=4955170&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01627.x In conclusion, haploidentical transplants are a good and promising alternative option for patients with childhood leukemia who lack an human leukocyte antigen‐matched donor (sibling or unrelated donor). (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955170 Wed, 22 Jun 2011 17:57:56 +0100 4955170 http://www.medworm.com/index.php?rid=4955169&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01629.x In this study, we analyzed the karyotype of 126 plasma cell leukemia (PCL) patients to identify the most frequently occurring imbalances and to design a model of karyotypic evolution. The Mitelman database of chromosome was searched and abnormal karyotypes were assessed. The main clones were analyzed and chromosomal gains and losses were used to design a model of genetic acquisition based on the calculation of a variable called time to occurrence. Our comprehensive study of genetic abnormalities of a large number of PCL karyotypes suggests that PCL is mainly characterized by the presence of whole chromosome losses as well as IgH rearrangements which is similar to that observed in non‐hyperdiploid multiple myeloma (MM). Temporal analysis suggests that most PCL have around 10 abnormalities... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955169 Wed, 22 Jun 2011 17:57:54 +0100 4955169 http://www.medworm.com/index.php?rid=4955168&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01626.x AbstractWe explored the role of CD38 and functionally associated molecular risk factors in a recently described chronic lymphocytic leukemia (CLL) nonobese diabetic/ severe combined immunodeficient xenograft model. Intravenous injection of peripheral blood mononuclear cells from 73 patients with CLL into 244 mice resulted in robust engraftment of leukemic cells into the murine spleens detected 4 wks after transplantation. Leukemic cell engraftment correlated significantly (P < 0.05) with markers reflecting disease activity, e.g., Binet stage and lymphocyte doubling time, and the expression of molecular risk factors including CD38, CD49d, ZAP‐70, and IgVH mutational status. Increased engraftment levels of CD38+ as compared to CD38− CLL cells could be attributed, in part, to leu... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955168 Wed, 22 Jun 2011 17:57:53 +0100 4955168 http://www.medworm.com/index.php?rid=4955167&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01617.x AbstractAutoimmune lymphoproliferative syndrome (ALPS) is a disorder of lymphocyte homeostasis. It is characterized by non‐malignant lymphoproliferation autoimmunity mostly directed toward blood cells and increased risk of lymphoma. Majority of patients with ALPS harbor heterozygous germline mutations in the gene for the TNF receptor‐family member Fas (CD 95, Apo‐1) which are inherited in an autosomal dominant fashion. Somatic Fas mutations are the second most common genetic etiology of ALPS. Additionally mutations in the genes encoding Fas‐ligand (FASLG), caspase 10 (CASP10) and caspase 8 (CASP8), NRAS and KRAS have been identified in a small number of patients with ALPS and related disorders. Approximately one‐third of patients with ALPS have yet unidentified defect. ALPS was i... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955167 Wed, 22 Jun 2011 17:57:53 +0100 4955167 http://www.medworm.com/index.php?rid=4955166&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01670.x In this study, a three‐month‐old boy with non‐consanguineous parents was referred for convulsions due to intracerebral haemorrhage. Standard coagulation tests revealed that the patient’s plasma prothrombin activity was 12%, while his father’s and mother’s levels were 55% and 70%, respectively. Analysis of the prothrombin gene revealed that this patient is a compound heterozygote for two missense mutations: one maternally‐inherited point mutation in the propeptide (p.Arg4Gln), and one paternally‐inherited mutation in the kringle‐2 (p.Arg220Pro) domain. Structural analysis was performed and confirmed that the resulting mutations were inferred to respectively affect the cleavage of the propeptide from the Gla domain, and the stability of the kringle‐2 domain, both resultin... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4955166 Wed, 22 Jun 2011 17:57:21 +0100 4955166 http://www.medworm.com/index.php?rid=5080766&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01668.x (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5080766 Wed, 15 Jun 2011 23:00:00 +0100 5080766 http://www.medworm.com/index.php?rid=4938770&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01667.x SummaryLenalidomide is an immunomodulatory agent with activity in a range of haematological cancers including chronic lymphocytic leukaemia (CLL). However, major questions remain concerning its effectiveness in patients with T‐cell depletion or deletion of TP53 on chromosome 17p. This case report provides insight into these questions by showing that lenalidomide/dexamethasone in combination can be highly effective as induction therapy for refractory 17p‐ CLL, that remission quality can be improved by subsequent maintenance with lenalidomide alone, and that the anti‐leukemic effects of lenalidomide and its stimulatory effects on non‐malignant B cells are preserved despite prolonged T‐cell depletion resulting from prior alemtuzumab/methylprednisolone. (Source: European Journal of H... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4938770 Wed, 15 Jun 2011 23:00:00 +0100 4938770 http://www.medworm.com/index.php?rid=4938771&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01666.x Conclusions:  PTX3 was superior to CRP in predicting complicated course of febrile neutropenia, but only when the effect of the underlying malignancy had been taken into account. (Source: European Journal of Haematology) European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=4938771 Tue, 14 Jun 2011 23:00:00 +0100 4938771 http://www.medworm.com/index.php?rid=5080767&cid=s_29467_19_f&fid=29467&url=http%3A%2F%2Fdx.doi.org%2F10.1111%252Fj.1600-0609.2011.01662.x AbstractFollowing 1‐yr deferasirox therapy in the ESCALATOR study, 57% of previously chelated patients with β‐thalassaemia achieved treatment success (maintenance of or reduction in liver iron concentration (LIC) vs. baseline LIC). Seventy‐eight per cent had dose increases at median of 26 wk, suggesting that 1‐yr results may not have reflected full deferasirox efficacy. Extension data are presented here. Deferasirox starting dose was 20 mg/kg/d (increases to 30/40 mg/kg/d permitted in the core/extension, respectively). Efficacy was primarily assessed by absolute change in LIC and serum ferritin. Overall, 231 patients received deferasirox in the extension; 67.4% (P < 0.0001) achieved treatment success. By the end of the extension, 66.2% of patients were receiving doses... European Journal of Haematology journals http://www.medworm.com/rss/comments.php?id=5080767 Fri, 10 Jun 2011 23:00:00 +0100 5080767