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 'American Journal of Hematology' source. Wed, 08 Feb 2012 17:41:56 +0100 http://www.medworm.com/index.php?rid=5668129&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23142 AbstractDuring the past 25 years, our knowledge concerning the pathogenesis, diagnostic strategies and treatment of von Willebrand disease (VWD) has increased significantly. Following the immunological differentiation of factor VIII (FVIII) and von Willebrand factor (VWF) in the 1970s and the cloning of the FVIII and VWF genes in the mid‐1980s, substantial progress has been made in our understanding of this, the most common inherited bleeding disorder. We now recognize that VWD represents a range of genetic diseases all with the clinical endpoint of increased mucocutaneous bleeding. The molecular pathology of Type 2 and Type 3 VWD is now comprehensively documented and involves rare sequence variants at the VWF locus. In contrast, the genetic causation of Type 1 disease remains incomplete... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5668129 Wed, 08 Feb 2012 05:00:00 +0100 5668129 http://www.medworm.com/index.php?rid=5668128&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23143 AbstractThromboembolism, including both venous and arterial events, occurs commonly amongst patients with cancer. The occurrence of thromboembolism has significant consequences for cancer patients, including direct and indirect associations with mortality, morbidity, requirement for long‐term anticoagulant therapy and consumption of healthcare resources. Recent studies have resulted in a better understanding of clinical risk factors and biomarkers of cancer‐associated thrombosis, and a risk assessment model incorporating both has now been validated in multiple settings. Thromboprophylaxis with either unfractionated heparin or low‐molecular‐weight heparins (LMWHs) has been shown to be safe and effective in high‐risk settings such as hospitalization for medical illness and the post... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5668128 Wed, 08 Feb 2012 05:00:00 +0100 5668128 http://www.medworm.com/index.php?rid=5668127&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23145 AbstractThe last decade has seen increased use of aggressive, catheter‐based methods of treating venous thromboembolism (DVT). In this article, we outline the risks, benefits, and uncertainties surrounding endovascular DVT therapies, describe clinical situations in which endovascular treatment options should reasonably be considered, and update the reader on new outcomes data that pertains to catheter‐based DVT interventions. Endovascular thrombolytic therapy is reasonable to perform for selected patients with DVT causing acute limb‐threatening circulatory compromise, acute IVC occlusion, or acute iliofemoral DVT for the purposes of limb salvage and relief of presenting DVT symptoms, and appears likely to prevent post‐thrombotic syndrome (PTS) in patients with proximal DVT. A multi... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5668127 Wed, 08 Feb 2012 05:00:00 +0100 5668127 http://www.medworm.com/index.php?rid=5668126&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23146 AbstractRecombinant DNA technology and protein engineering are creating hope that we can address ongoing challenges in hemophilia care such as reducing the costs of therapy, increasing the availability to the developing world, and improving the functional properties of these proteins. Technological advances to improve the half‐life of recombinant clotting factors have brought long acting clotting factors for hemophilia replacement therapy closer to reality. Preclinical and clinical trial results are reviewed as well as the potential benefits and risks of these novel therapies. Am. J. Hematol., 2012. © 2012 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5668126 Wed, 08 Feb 2012 05:00:00 +0100 5668126 http://www.medworm.com/index.php?rid=5668125&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23147 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5668125 Wed, 08 Feb 2012 05:00:00 +0100 5668125 http://www.medworm.com/index.php?rid=5668124&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23148 AbstractPOEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) syndrome is a rare multisystemic disease associated with plasma cell dyscrasia and increased serum or plasma Vascular Endothelial Growth Factor (VEGF) levels, the latter likely responsible for several POEMS syndrome manifestations. Whereas peripheral neuropathy is the main neurological feature and a mandatory diagnostic criterium, central nervous system involvement is less common except for papilledema and stroke. We recently reported the frequent occurrence at brain MRI of cranial pachymeningeal involvement in a series of POEMS syndrome patients. Meningeal histopathology revealed hyperplasia of meningothelial cells, neovascularisation and obstructive vessel remodelling without inflammatory si... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5668124 Wed, 08 Feb 2012 05:00:00 +0100 5668124 http://www.medworm.com/index.php?rid=5668123&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23149 Conclusions:Pediatric‐inspired regimens are superior to conventional‐adult chemotherapy in AYA ALL patients. Further randomized controlled studies to investigate this approach in adult ALL patients are warranted. Am. J. Hematol., 2012. © 2012 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5668123 Wed, 08 Feb 2012 05:00:00 +0100 5668123 http://www.medworm.com/index.php?rid=5668122&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23150 In conclusion, NEAM conditioning with auto‐SCT demonstrated considerable efficacy with modest toxicity in patients with chemosensitive aggressive NHL. Am. J. Hematol., 2012. © 2012 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5668122 Wed, 08 Feb 2012 05:00:00 +0100 5668122 http://www.medworm.com/index.php?rid=5668121&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23151 AbstractA few warfarin pharmacogenetic dosing algorithms have been proposed, based on multiethnic or homogeneous populations, to estimate warfarin therapeutic doses. However, it remains to be proven which algorithm is accurate in predicting warfarin dose in Chinese people. We selected eight warfarin dose predictive pharmacogenetic algorithms and retrospectively assessed the predictive accuracy of each algorithm in a total of 368 eligible outpatients by comparing the actual stable therapeutic dose to the dose predicted by the algorithm. Our results showed that a high level of performance was demonstrated by three algorithms, Gage et al., Anderson et al. and Wu et al., having a similar performance in coefficient of determination (R2) and percentage of patients predicted dose within 20% of ac... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5668121 Wed, 01 Feb 2012 05:00:00 +0100 5668121 http://www.medworm.com/index.php?rid=5649309&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23140 In conclusion, analysis of this large series of pediatric patients with primary myelofibrosis demonstrates distinct clinical, hematologic, bone marrow, and molecular features from adult patients. Am. J. Hematol., 2012. © 2012 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5649309 Wed, 01 Feb 2012 05:00:00 +0100 5649309 http://www.medworm.com/index.php?rid=5649313&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23137 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5649313 Tue, 31 Jan 2012 05:00:00 +0100 5649313 http://www.medworm.com/index.php?rid=5649312&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23138 AbstractEssential Thrombocythaemia (ET) is a rare type of myeloproliferative neoplasm (MPN) characterized by clonal expansion of the megakaryocyte and platelet lineage. Here, we describe a novel mutation (Y252H) in the thrombopoietin (TPO) receptor, or MPL, in a JAK2 mutation‐negative ET patient. The bone marrow examination revealed increased numbers of dysmorphic megakaryocytes with focal clustering. The x‐inactivation pattern suggested clonal expansion of hematopoietic cells in the bone marrow. Furthermore we found that the patient's bone marrow cells were hypersensitive to TPO in generating megakaryocyte colonies in vitro. More importantly, we demonstrated that this MPL Y252H mutant confers increased TPO/MPL‐mediated cell growth and increased cell survival upon cytokine withdrawal... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5649312 Tue, 31 Jan 2012 05:00:00 +0100 5649312 http://www.medworm.com/index.php?rid=5649311&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23139 AbstractThe new oral anticoagulants are rapidly replacing warfarin for several indications. In contrast to warfarin, which lowers the functional levels of all of the vitamin K‐dependent clotting factors, the new agents target either factor Xa or thrombin. With targeted inhibition of coagulation, the new oral anticoagulants have pharmacological, biochemical and clinical features distinct from those of warfarin. Focusing on laboratory perspectives, this paper compares and contrasts the pharmacological and biochemical properties of the new oral anticoagulants with those of warfarin and uses this information to speculate on the underlying mechanisms responsible for the clinical features that differentiate the new agents from warfarin. Am. J. Hematol., 2012. © 2012 Wiley‐Liss, Inc. (Source... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5649311 Tue, 31 Jan 2012 05:00:00 +0100 5649311 http://www.medworm.com/index.php?rid=5649310&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23141 AbstractIn AL amyloidosis prognosis depends on the severity of heart dysfunction which is best assessed by natriuretic peptides (BNP and NT‐proBNP). However, their clearance relies on glomerular filtration rate (GFR) and their concentration increases with renal failure. We evaluated the diagnostic and prognostic performance of NT‐proBNP and BNP in 248 patients with AL amyloidosis with different degrees of renal failure.Patients were grouped according to GFR. Group 1 comprised 109 patients with GFR ≥60 mL/min/1.73m2, Group 2, 77 subjects with GFR <60 and ≥15 mL/min/1.73m2, and Group 3, 62 patients with GFR <15 mL/min/1.73m2. The ability of natriuretic peptide to detect heart involvement and to predict survival in the 3 groups was assessed.Decreasing eGFR required higher cutoff... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5649310 Tue, 31 Jan 2012 05:00:00 +0100 5649310 http://www.medworm.com/index.php?rid=5639130&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23136 AbstractCardioembolic strokes account for one‐sixth of all strokes and are an important potentially preventable cause of morbidity and mortality. Vitamin K antagonists (e.g., warfarin) are effective for the prevention of cardioembolic stroke in patients with atrial fibrillation (AF) and in those with mechanical heart valves but because of their inherent limitations are underutilized and often suboptimally managed. Antiplatelet therapies have been the only alternatives to warfarin for stroke prevention in AF but although they are safer and more convenient they are much less efficacious. The advent of new oral anticoagulant drugs offers the potential to reduce the burden of cardioembolic stroke by providing access to effective, safe and more convenient therapies. New oral anticoagulants ha... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5639130 Sun, 29 Jan 2012 22:11:34 +0100 5639130 http://www.medworm.com/index.php?rid=5629907&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23131 AbstractVenous thromboembolism (VTE) is an increasingly common complication encountered in tertiary care pediatric settings. The purpose of this review is to summarize the epidemiology, current and emerging pharmacotherapeutic options, and management of this disease. Over 70% of VTE occur in children with chronic diseases. Although they are seen in children of all ages, adolescents are at greatest risk. Pediatric VTE is associated with an increased risk of in‐hospital mortality; recurrent VTE and post‐thrombotic syndrome are commonly seen in survivors. In recent years, anticoagulation with low molecular weight heparin has emerged as the mainstay of therapy, but compliance is limited by its onerous subcutaneous administration route. New anticoagulants either already approved for use in ... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5629907 Thu, 26 Jan 2012 22:30:54 +0100 5629907 http://www.medworm.com/index.php?rid=5629908&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23132 AbstractManagement of adults with primary immune thrombocytopenia (ITP) has changed dramatically in the past 10 years. New regimens of corticosteroids for 1st‐line treatment have been introduced and are currently being evaluated in a randomized clinical trial. Many patients may not have durable remissions with initial corticosteroid regimens and may require additional, 2nd‐line, treatment. For these patients, rituximab has been increasingly used, as it has for other autoimmune disorders, and new thrombopoietin (TPO)‐receptor agonists have been developed. Although splenectomy was the first effective and remains the most effective treatment for ITP, inducing durable complete remissions in 66% of patients, rituximab and TPO‐receptor agonists are now additional options for 2nd‐line t... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5629908 Wed, 25 Jan 2012 05:00:00 +0100 5629908 http://www.medworm.com/index.php?rid=5610092&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23130 We describe an AMKL‐DS case who suffered from severe pulmonary fibrosis and required tracheostomy. We identified the unique GATA1 mutation site in the leukemic cells of the patient and succeeded in performing real time quantitative polymerase chain reaction (RQ‐PCR) for measurement of minimal residual disease (MRD). To prevent fatal respiratory infection during myelosuppression with conventional aggressive chemotherapy, the patient basically received 12 courses consisting of 14 days of ultra low‐dose cytarabine (10mg/m2/dose, twice per day) for 12 months using the GATA1 mutation to monitor MRD and has been in complete remission for more than 23 months after achieving her 1st complete remission. Ultra low‐dose cytarabine treatment using GATA1 mutation by RQ‐PCR to monitor MRD is a... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610092 Fri, 20 Jan 2012 22:06:16 +0100 5610092 http://www.medworm.com/index.php?rid=5610094&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23128 AbstractVenous thromboembolism (VTE) is a chronic disease with a 30% ten‐year recurrence rate. The highest incidence of recurrence is in the first 6 months. Active cancer significantly increases the hazard of early recurrence, and the proportions of time on standard heparin with an APTT≥0.2 anti‐Xa U/mL, and on warfarin with an INR≥2.0, significantly reduce the hazard. The acute treatment duration does not affect recurrence risk after treatment is stopped. Independent predictors of late recurrence include increasing patient age and body mass index, leg paresis, active cancer and other persistent VTE risk factors, idiopathic VTE, antiphospholipid antibody syndrome, antithrombin, protein C or protein S deficiency, hyperhomocysteinemia and a persistently increased plasma fibrin D‐di... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610094 Fri, 20 Jan 2012 05:00:00 +0100 5610094 http://www.medworm.com/index.php?rid=5610093&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23129 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610093 Fri, 20 Jan 2012 05:00:00 +0100 5610093 http://www.medworm.com/index.php?rid=5610106&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23117 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610106 Thu, 19 Jan 2012 05:00:00 +0100 5610106 http://www.medworm.com/index.php?rid=5610105&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23118 AbstractMutations in GBA1 gene result in defective acid β‐glucosidase and the complex phenotype of Gaucher disease (GD) related to the accumulation of glucosylceramide‐laden macrophages. The phenotype is highly variable even among patients harboring identical GBA1 mutations. We hypothesized that modifier gene(s) underlie phenotypic diversity in GD and performed a GWAS study in Ashkenazi Jewish patients with type 1 GD (GD1), homozygous for N370S mutation. Patients were assigned to mild, moderate or severe disease category using composite disease severity scoring systems. Whole‐genome genotyping for >500,000 SNPs was performed to search for associations using OQLS algorithm in 139 eligible patients. Several SNPs in linkage disequilibrium within the CLN8 gene locus were associated w... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610105 Thu, 19 Jan 2012 05:00:00 +0100 5610105 http://www.medworm.com/index.php?rid=5610104&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23119 Conclusion:The risk of TE in this population was not negligible. Given the frequent observation of subtherapeutic INR levels when monitoring vitamin K antagonists, this finding warrants additional investigation to improve the management of these patients. Am. J. Hematol., 2012. © 2012 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610104 Thu, 19 Jan 2012 05:00:00 +0100 5610104 http://www.medworm.com/index.php?rid=5610103&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23120 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610103 Thu, 19 Jan 2012 05:00:00 +0100 5610103 http://www.medworm.com/index.php?rid=5610102&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23121 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610102 Thu, 19 Jan 2012 05:00:00 +0100 5610102 http://www.medworm.com/index.php?rid=5610101&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23122 In conclusion, LMWH given at fixed sub‐therapeutic is a feasible and relatively safe approach for bridging therapy in cancer patients on long‐term VKA. Am. J. Hematol., 2012. © 2012 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610101 Thu, 19 Jan 2012 05:00:00 +0100 5610101 http://www.medworm.com/index.php?rid=5610100&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23123 We report here the identification of two new heterozygous HIF2A missense mutations, M535T and F540L, both associated with erythrocytosis. Met‐535 has previously been identified as a residue mutated in other patients with erythrocytosis, although the mutation of this particular residue to Thr has not been reported. In contrast, Phe‐540 has not been reported as a residue mutated in erythrocytosis, and we present evidence here that this mutation impairs interaction of HIF‐2α with both VHL and PHD2. Am. J. Hematol., 2012. © 2012 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610100 Thu, 19 Jan 2012 05:00:00 +0100 5610100 http://www.medworm.com/index.php?rid=5610098&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23124 AbstractBleeding disorders commonly result from deficiencies or defects in von Willebrand factor (VWF), platelets, coagulation factors, or fibrinolytic proteins. The primary goal of our study was to assess current North American coagulation laboratory practices for diagnosing bleeding disorders, using an on‐line patterns‐of‐practice survey of diagnostic laboratory members of the North American Specialized Coagulation Laboratory Association (NASCOLA). The survey examined laboratory approaches to evaluating bleeding disorders, with specific questions about the tests and test panels offered and compliance to recent guideline recommendations on diagnosing von Willebrand disease (VWD) and platelet function disorders.All laboratories responding to the survey performed a prothrombin time/in... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610098 Thu, 19 Jan 2012 05:00:00 +0100 5610098 http://www.medworm.com/index.php?rid=5610097&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23125 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610097 Thu, 19 Jan 2012 05:00:00 +0100 5610097 http://www.medworm.com/index.php?rid=5610096&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23126 AbstractAlthough treatment with plasma exchange increased the survival of patients with thrombotic thrombocytopenia purpura (TTP) to 80% in the 1980s, no further increase of survival occurred over the next 20 years. However more consistent use of adjuvant treatment with corticosteroids and rituximab in recent years has begun to further increase survival as well as decrease the frequency of relapse. With adjuvant treatment, durable remissions can be achieved more quickly, requiring fewer days of plasma exchange. Fewer days of plasma exchange have resulted in fewer complications, such as central venous catheter‐related systemic infections. Future potential options for adjuvant treatment, recombinant ADAMTS13 to correct severe ADAMTS13 deficiency and agents to block von Willebrand factor‐... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610096 Thu, 19 Jan 2012 05:00:00 +0100 5610096 http://www.medworm.com/index.php?rid=5610095&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23127 AbstractTwo decades of research into heparin‐induced thrombocytopenia (HIT) permit a personal historical perspective on this fascinating syndrome. Previously, the frequency of HIT was unknown, although complicating thrombosis was believed to be rare and primarily arterial. The opportunity to apply a remarkable test for “HIT antibodies”—the 14C‐serotonin‐release assay (SRA)—to serial plasma samples obtained during a clinical trial of heparin thromboprophylaxis, provided insights into the peculiar nature of HIT, such as: its prothrombotic nature—including its strong association with venous thrombosis (RR=11.6 [95%CI, 6.4‐20.8; p<0.0001); its more frequent occurrence with unfractionated versus low‐molecular‐weight heparin; the “iceberg” model, which states that am... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5610095 Thu, 19 Jan 2012 05:00:00 +0100 5610095 http://www.medworm.com/index.php?rid=5598828&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22248 AbstractDisease Overview: Immunoglobulin (Ig) light chain amyloidosis is a clonal but nonproliferative plasma cell disorder in which fragments of an Ig light chain are deposited in tissues. The clinical features depend on the organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, and peripheral/autonomic neuropathy.Diagnosis: Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for diagnosis. Invasive organ biopsy is not required because amyloid deposits can be found in bone marrow biopsy or subcutaneous fat aspirate in 85% of patients. Prognosis: N‐terminal pro–brain natriuretic peptide and serum troponin T values are used to classify patients into three groups of approximately equal ... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5598828 Tue, 17 Jan 2012 23:03:32 +0100 5598828 http://www.medworm.com/index.php?rid=5583453&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23115 AbstractMantle cell lymphoma (MCL) and chronic lymphocytic leukemia (CLL) are mature CD5+ B‐cell malignancies with different biological/clinical characteristics. We recently reported an association between different prognostic subgroups of CLL (i.e. IGHV mutated and unmutated) and genomic methylation pattern. However, the relationship between DNA methylation and prognostic markers, such as the proliferation gene expression signature, has not been investigated in MCL. We applied high‐resolution methylation microarrays (27,578 CpG sites) to assess the global DNA methylation profiles in 20 MCL (10 each with high/low proliferation signature) and 30 CLL (15 poor‐prognostic IGHV unmutated subset #1 and 15 good‐prognostic IGHV mutated subset #4) samples. Notably, MCL and each CLL subset d... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5583453 Wed, 11 Jan 2012 05:00:00 +0100 5583453 http://www.medworm.com/index.php?rid=5639132&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23113 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5639132 Thu, 05 Jan 2012 05:00:00 +0100 5639132 http://www.medworm.com/index.php?rid=5639131&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23114 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5639131 Thu, 05 Jan 2012 05:00:00 +0100 5639131 http://www.medworm.com/index.php?rid=5561311&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23107 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5561311 Thu, 05 Jan 2012 05:00:00 +0100 5561311 http://www.medworm.com/index.php?rid=5561310&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23114 AbstractT2* magnetic resonance imaging (MRI) provides rapid quantification of liver iron content (LIC). The reciprocal of T2* is directly proportional to iron and has been calibrated against LIC. There has, however, been few independent validation of the T2* method in a clinical setting. In 100 MRI studies on 75 pediatric patients being investigated for liver iron overload, we assess the accuracy and reproducibility of T2*‐measured LIC, using regulatory approved T2‐based FerriScan® for reference measurements. Results from independent analyses by two observers demonstrated robust inter‐ and intra‐observer agreement (intraclass correlation coefficient (ICC) = 0.99 and 1.0, respectively). T2*‐measured and reference LIC were strongly correlated (r = 0.94, P < 0.0001), with a regr... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5561310 Thu, 05 Jan 2012 05:00:00 +0100 5561310 http://www.medworm.com/index.php?rid=5561309&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23113 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5561309 Thu, 05 Jan 2012 05:00:00 +0100 5561309 http://www.medworm.com/index.php?rid=5583452&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23116 AbstractHeat shock protein (HSP) 70 and HSP90 are released by primary human acute myeloid leukemia (AML) cells during stress‐induced spontaneous in vitro apoptosis. The AML cells also show constitutive release of several cytokines, and the systemic serum levels of several soluble mediators are altered in patients with untreated AML. In the present study we investigated serum levels of HSP70/HSP90 and the serum cytokine profiles of patients with untreated AML and patients receiving AML‐stabilizing palliative treatment based on all‐trans retinoic acid (ATRA) plus valproic acid. Patients with untreated AML showed increased HSP90 levels and a distinct serum cytokine profile compared with healthy controls, and low pre‐therapy HSP90 levels were associated with a prolonged survival during... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5583452 Sun, 01 Jan 2012 05:00:00 +0100 5583452 http://www.medworm.com/index.php?rid=5561308&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23112 AbstractRomidepsin is the second histone deacytelase inhibitor (HDACi) approved for the treatment of advanced stages of cutaneous T cell lymphoma (CTCL). Recent in vitro data suggests that HDACi suppress immune function although these findings have not been confirmed in patients. Thus, we serially examined the cellular immune function of eight CTCL patients undergoing treatment with three cycles of romidepsin. We measured the patient's natural killer (NK) and dendritic cell (DC) function, and performed an in vitro TUNEL assay to measure cellular apoptosis. Patients' NK cell cytolytic activity decreased from baseline to the third cycle of treatment (P=.018) but stimulation with a TLR agonist increased this activity (P=.018). At baseline a TLR agonist could both activate patients' DC (P=.043... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5561308 Sun, 01 Jan 2012 05:00:00 +0100 5561308 http://www.medworm.com/index.php?rid=5553054&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23111 AbstractNeonatal alloimmune thrombocytopenia (NAIT) is the most common cause of severe thrombocytopenia and intracranial hemorrhage (ICH) in the perinatal period. While the gold standard for making a diagnosis of NAIT is detection of an HPA‐specific antibody in maternal serum, together with identifying an incompatibility between the parents for the cognate HPA antigen, platelet genotyping is the gold standard method for HPA typing. Platelet genotyping is critical in screening at‐risk fetuses for the presence of the HPA corresponding to the maternal antibody. In addition, platelet genotyping may play a role in population screening to identify women at risk for sensitization, and thus, fetuses at risk for NAIT. The most commonly used methods of platelet genotyping are sequence‐specific... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5553054 Sat, 31 Dec 2011 20:30:14 +0100 5553054 http://www.medworm.com/index.php?rid=5649314&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23110 AbstractAnemia of chronic disease (ACD) or anemia of inflammation is prevalent in patients with chronic infection, autoimmune disease, cancer, and chronic kidney disease. ACD is associated with poor prognosis and lower quality of life. Management of ACD using intravenous iron and erythropoiesis stimulating agents are ineffective for some patients and are not without adverse effects, driving the need for new alternative therapies. Recent advances in our understanding of the molecular mechanisms of iron regulation reveal that increased hepcidin, the iron regulatory hormone, is a key factor in the development of ACD. In this review, we will summarize the role of hepcidin in iron homeostasis, its contribution to the pathophysiology of ACD, and novel strategies that modulate hepcidin and its ta... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5649314 Thu, 29 Dec 2011 05:00:00 +0100 5649314 http://www.medworm.com/index.php?rid=5553060&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23104 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5553060 Thu, 29 Dec 2011 05:00:00 +0100 5553060 http://www.medworm.com/index.php?rid=5553059&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23105 AbstractChronic transfusions are recommended for children with sickle cell anemia (SCA) and abnormal transcranial Doppler (TCD) velocities (≥ 200 cm/sec) to help prevent the occurrence of a primary stroke.[1] The goal is usually to maintain the sickle hemoglobin concentration (HbS) <30%, however, this goal is often difficult to achieve in clinical practice. The NHLBI‐sponsored trial “TCD With Transfusions Changing to Hydroxyurea (TWiTCH)” will compare standard therapy (transfusions) to alternative therapy (hydroxyurea) for the reduction of primary stroke risk in this patient population. Transfusions will be given according to current transfusion practices at participating sites. To determine current academic community standards for primary stroke prophylaxis in children with SCA... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5553059 Thu, 29 Dec 2011 05:00:00 +0100 5553059 http://www.medworm.com/index.php?rid=5553058&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23106 AbstractDespite advances in our understanding of the pathophysiology of thrombotic thrombocytopenic purpura (TTP), there remains significant room for improvement in the treatment of acute TTP. A novel approach to the treatment of TTP using ARC1779 to target the A1‐domain of von Willebrand Factor (VWF) to prevent the formation of microthombi has been developed. Preliminary data suggests that blockade of the A1‐domain of VWF by ARC1779 can inhibit VWF activity, resulting in clinically significant improvements in the platelet count and lactate dehydrogenase(1‐5). ARC1779 is a nucleic acid macromolecule, or aptamer, that inhibits the prothrombotic function of VWF by binding to the A1‐domain of VWF, blocking its interaction with the platelet GPIb receptor. It has been hypothesized that ... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5553058 Thu, 29 Dec 2011 05:00:00 +0100 5553058 http://www.medworm.com/index.php?rid=5553057&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23108 AbstractIn patients with recurrent Hodgkin or non‐Hodgkin's lymphoma, autologous stem cell transplantation (ASCT) can offer potential for cure or long term remission. Because of potential toxicity, elderly patients are usually not considered candidates, but data regarding tolerability and efficacy in this group are lacking. The transplant database at Weill Cornell Medical College was reviewed to identify patients with lymphoma undergoing ASCT at age 69 or greater. Clinical data and comorbidities were correlated with outcome. Twenty‐one patients were identified. Sixteen of 19 evaluable patients (76%) achieved complete remission following ASCT, while 2 patients died before response assessment. Median progression‐free survival following ASCT was 8 months and median overall survival was ... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5553057 Thu, 29 Dec 2011 05:00:00 +0100 5553057 http://www.medworm.com/index.php?rid=5553056&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23109 This study retrospectively evaluated the long‐term safety and efficacy of a conditioning regimen consisting of total body irradiation (TBI; 12 Gy), cyclophosphamide (CY; 60 mg/kg, 2 doses), and high‐dose cytarabine (Ara‐C; 2 g/m2; 4 doses) for patients with ALL. Fifty‐five patients (median age: 31 years old) were evaluated. Stem cells were from human leukocyte antigen‐identical siblings in 22 patients and from alternative donors in 33. There were no cases of early death before engraftment, and 100‐day transplant‐related mortality was 7.3%. With a median follow‐up period of 9.6 years, 5‐year overall and disease‐free survival were 63.2% (95% CI: 46.5‐79.9%) and 63.6% (95% CI: 47.1‐80.1%) in patients with complete remission, respectively, both of which were significant... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5553056 Thu, 29 Dec 2011 05:00:00 +0100 5553056 http://www.medworm.com/index.php?rid=5553055&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.23110 AbstractAnemia of Chronic Disease (ACD) or Anemia of Inflammation (AI) is prevalent in patients with chronic infection, autoimmune disease, cancer and chronic kidney disease. ACD is associated with poor prognosis and lower quality of life. Management of ACD using intravenous iron and erythropoiesis stimulating agents (ESAs) are ineffective for some patients and are not without adverse effects, driving the need for new alternative therapies. Recent advances in our understanding of the molecular mechanisms of iron regulation reveal that increased hepcidin, the iron regulatory hormone, is a key factor in the development of ACD. In this review, we will summarize the role of hepcidin in iron homeostasis, its contribution to the pathophysiology of ACD, and novel strategies that modulate hepcidin... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5553055 Thu, 29 Dec 2011 05:00:00 +0100 5553055 http://www.medworm.com/index.php?rid=5516067&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22246 AbstractDisease Overview: Acute myeloid leukemia (AML) results from accumulation of abnormal immature cells in the marrow. These cells interfere with normal hematopoiesis can escape into the blood and infiltrate lung and CNS. The most common cause of death is bone marrow failure. It is likely that many different mutations and/or epigenetic aberrations can produce the same disease, with these differences responsible for the very variable response to therapy, which is AML's principal clinical feature.Diagnosis: This rests on demonstration that the marrow or blood has >20% blasts of myeloid lineage. Blast lineage is assessed by multiparameter flow cytometry with CD33 and CD13 being surface markers typically expressed by myeloid blasts. It should be realized that clinical/prognostic conside... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5516067 Mon, 19 Dec 2011 10:31:00 +0100 5516067 http://www.medworm.com/index.php?rid=5516066&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22237 AbstractDisease overview: Multiple myeloma accounts for ∼10% of all hematologic malignancies.Diagnosis: The diagnosis requires 10% or more clonal plasma cells on bone marrow examination or a biopsy proven plasmacytoma plus evidence of end‐organ damage felt to be related to the underlying plasma‐cell disorder.Risk stratification: Patients with 17p deletion, t(14;16), t(14;20), or high‐risk gene expression profiling signature have high‐risk myeloma. Patients with t(4;14) translocation, karyotypic deletion 13, or hypodiploidy are considered to have intermediate‐risk disease. All others are considered to have standard‐risk myeloma.Risk‐adapted therapy: Standard‐risk patients are treated with nonalkylator‐based therapy such as lenalidomide plus low‐dose dexamethasone (Rd) ... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5516066 Mon, 19 Dec 2011 10:30:59 +0100 5516066 http://www.medworm.com/index.php?rid=5516059&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22275 AbstractIn HIV‐positive patients with non‐Hodgkin lymphoma (NHL), no benefit of adding rituximab to chemotherapy was seen in a randomized controlled trial (RCT). We performed a meta‐analysis of prospective studies to ascertain outcomes in HIV‐positive NHL patients treated with chemotherapy (chemo) versus rituximab and chemo (R‐chemo). A literature search through September 2011 was performed using the key search “(HIV OR AIDS) AND lymphoma”. The main outcomes were overall response rate (ORR), complete response rate (CRR) and 2‐year overall survival (OS) and are reported as non‐adjusted odds ratio (OR). We identified 15 prospective studies including 1,060 HIV‐positive NHL patients, 675 treated with chemo and 385 with R‐chemo. There was a higher proportion of HAART in R�... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5516059 Mon, 19 Dec 2011 10:28:57 +0100 5516059 http://www.medworm.com/index.php?rid=5583454&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22265 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5583454 Sat, 17 Dec 2011 05:00:00 +0100 5583454 http://www.medworm.com/index.php?rid=5516065&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22265 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5516065 Sat, 17 Dec 2011 05:00:00 +0100 5516065 http://www.medworm.com/index.php?rid=5516064&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22270 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5516064 Sat, 17 Dec 2011 05:00:00 +0100 5516064 http://www.medworm.com/index.php?rid=5516063&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22271 AbstractSickle Cell Trait (HbAS), the heterozygous state for the sickle hemoglobin beta globin gene is carried by as many as 100 million individuals including up to 25% of the population in some regions of the world.(1) Persons with HbAS have some resistance to falciparum malaria infection in early childhood (2,3) and as a result individuals with HbAS living in malarial endemic regions of Africa have a survival advantage over individuals with HbAA. Reports from the US emphasize possible health risks for individuals with HbAS including increased incidence of renal failure and malignancy, thromboembolic disorders, splenic infarction as a high altitude complication, and exercise‐related sudden death. The National Heart, Lung, and Blood Institute, National Institutes of Health convened a wor... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5516063 Sat, 17 Dec 2011 05:00:00 +0100 5516063 http://www.medworm.com/index.php?rid=5516062&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22272 AbstractThe introduction of tyrosine kinase inhibitors (TKIs), starting with Imatinib and followed by second and third generation TKIs, has significantly changed the clinical management of Chronic Myeloid Leukaemia (CML) patients. Despite their unprecedented clinical success, a proportion of patients fail to achieve complete cytogenetic remission by 12 months of treatment (primary resistance) while others experience progressive resistance after an initial response (secondary resistance). BCR‐ABL1 kinase domain (KD) mutations have been detected in a proportion of patients at the time of treatment failure, and therefore their identification and monitoring plays an important role in therapeutic decisions particularly when switching TKIs. When monitoring KD mutations in a clinical laboratory... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5516062 Sat, 17 Dec 2011 05:00:00 +0100 5516062 http://www.medworm.com/index.php?rid=5516061&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22273 AbstractA total of 149 patients with multiple myeloma (MM) who received allogeneic hematopoietic stem cell transplantation (allo‐HCT) with myeloablative (MAC; n=38) or reduced‐intensity conditioning (RIC; n=110) regimens at MD Anderson Cancer Center were evaluated. Of the total, 120 (81%) patients had relapsed or had refractory disease. Median age of MM patients was 50 (28‐70) years with a follow‐up time of 28.5(3‐164) months. The 100‐day and 5‐year treatment related mortality (TRM) rates were 17% and 47%, respectively. TRM was significantly lower with RIC regimens (13%) vs. 29% for MAC at 100 days (p=0.012). The cumulative incidence of grade II‐IV acute graft‐versus‐host disease (GVHD) was 35% and chronic GVHD was 46%. PFS and OS at 5 years were 15% and 21%, respective... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5516061 Sat, 17 Dec 2011 05:00:00 +0100 5516061 http://www.medworm.com/index.php?rid=5516060&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22274 AbstractPlatelets are activated in sickle cell disease (SCD), and particularly during vaso‐occlusive episodes (VOE). Thrombospondin‐1 (TSP1), a major secretory product of activated platelets, is increased in the circulation in VOE and binds to sickle RBC promoting vascular adhesion. Thus, we hypothesized that TSP1 may represent a plasma biomarker of disease severity in SCD. We tested the plasma collected from patients in steady state (n=27) and VOE (n=14), as well as healthy controls (n=17) at the University of Pittsburgh Medical Center (UPMC), and from patients in steady state enrolled in the walk‐PHaSST clinical trial (n=483). We found that TSP1 levels were increased in VOE in the UPMC cohort. Among steady‐state patients at UPMC, TSP1 values correlated positively with lifetime hi... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5516060 Sat, 17 Dec 2011 05:00:00 +0100 5516060 http://www.medworm.com/index.php?rid=5545204&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22267 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5545204 Sat, 03 Dec 2011 05:00:00 +0100 5545204 http://www.medworm.com/index.php?rid=5526254&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22266 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5526254 Sat, 03 Dec 2011 05:00:00 +0100 5526254 http://www.medworm.com/index.php?rid=5469823&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22266 AbstractDapsone is an antibacterial sulfonamide with anti‐inflammatory property which showed therapeutic activity in patients with immune thrombocytopenia (ITP) (1‐6); the activity in patients who showed refractoriness to rituximab is unknown. We evaluated the effect of dapsone in 20 consecutive adult patients, median age 51 years, with primary ITP previously treated at least with steroids and rituximab. Median baseline platelet count was 19 × 109/L, the median interval between diagnosis of ITP and dapsone therapy was 46 months. Response (platelet count = 30 × 109/L) and complete response (platelet count = 100 × 109/L) were 55% and 20%, respectively; median time to response was 1 month. All responders were able to interrupt any other specific anti ITP treatment. The median duration ... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5469823 Sat, 03 Dec 2011 05:00:00 +0100 5469823 http://www.medworm.com/index.php?rid=5469822&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22267 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5469822 Sat, 03 Dec 2011 05:00:00 +0100 5469822 http://www.medworm.com/index.php?rid=5469821&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22268 AbstractIntensive consolidation chemotherapy for acute myeloid leukemia (AML) patients in complete remission is being increasingly administered on an outpatient basis. While this approach has been found to be safe and feasible in younger patients [1‐6], its safety in older patients remains unknown. We therefore undertook an evaluation of outpatient‐based consolidation chemotherapy in older AML patients, and compared results to younger patients treated at the same institution over the same time period. The overall rate of readmission was approximately 50%, mostly for infections, with mean admission duration of 2 weeks. The overall mortality rate was 2.2%. Readmission rates and duration of readmission were somewhat higher in older patients, but infection rate, intensive care (ICU) admiss... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5469821 Sat, 03 Dec 2011 05:00:00 +0100 5469821 http://www.medworm.com/index.php?rid=5469820&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22269 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5469820 Thu, 01 Dec 2011 05:00:00 +0100 5469820 http://www.medworm.com/index.php?rid=5450099&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22264 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5450099 Mon, 28 Nov 2011 10:40:14 +0100 5450099 http://www.medworm.com/index.php?rid=5526251&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22264 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5526251 Sun, 27 Nov 2011 05:00:00 +0100 5526251 http://www.medworm.com/index.php?rid=5442151&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22258 In conclusion, and consistent with previous reports, the natural history of patients with t(16:16)(p13;q22) is similar to that of classic patients with inv16 AML and therefore should be treated similarly. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442151 Fri, 25 Nov 2011 10:30:45 +0100 5442151 http://www.medworm.com/index.php?rid=5526252&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22263 AbstractNon‐Hodgkin lymphoma (NHL) represents a heterogenous group of neoplasias originating from lymphoid cells. Increased angiogenesis and expression of Vascular Endothelial Growth Factor (VEGF) and its receptors (VEGFR) have been found to be associated with NHL disease progression. Increase in VEGF and other cytokines stimulate signaling cascades, including the Ras/Raf/Mek/Erk pathway, resulting in increased proliferation and decreased apoptosis. Here, we report the in vitro antilymphoma activity of sorafenib, an inhibitor of VEGFR and Raf kinase. Sorafenib induced potent cytotoxicity in NHL cell lines and patient samples. This induction of cytotoxicity was associated with a corresponding increase in apoptotic cell death. Mechanism of action of sorafenib was investigated in follicular... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5526252 Wed, 23 Nov 2011 05:00:00 +0100 5526252 http://www.medworm.com/index.php?rid=5442156&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22263 AbstractNon‐Hodgkin lymphoma (NHL) represents a heterogenous group of neoplasias originating from lymphoid cells. Increased angiogenesis and expression of Vascular Endothelial Growth Factor (VEGF) and its receptors (VEGFR) have been found to be associated with NHL disease progression. Increase in VEGF and other cytokines stimulate signaling cascades including the Ras/Raf/Mek/Erk pathway resulting in increased proliferation and decreased apoptosis. Here, we report the in vitro anti‐lymphoma activity of sorafenib, an inhibitor of VEGFR and Raf kinase. Sorafenib induced potent cytotoxicity in NHL cell lines and patient samples. This induction of cytotoxicity was associated with a corresponding increase in apoptotic cell death. Mechanism of action of sorafenib was investigated in follicula... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442156 Wed, 23 Nov 2011 05:00:00 +0100 5442156 http://www.medworm.com/index.php?rid=5442155&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22262 AbstractESA therapy can increase hemoglobin, decrease blood transfusions, and improve quality of life in patients with chemotherapy induced anemia (CIA). Despite its benefits, ESA therapy increases the risk of venous thromboembolism (VTE) in cancer patients by 50% and can also cause iron restricted erythropoiesis in CIA patients, which may augment the tendency to develop VTE. We postulated that thrombocytosis, a risk factor for VTE in cancer patients, in CIA patients on ESA therapy might be a result of ESA induced iron restricted erythropoiesis. We performed a retrospective analysis of 187 CIA patients who were randomized to receive weekly Epoetin and IV ferric gluconate, oral ferrous sulfate, or no iron for 8 weeks. Nineteen patients experienced 29 VTEs, and patients, whose platelets incr... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442155 Wed, 23 Nov 2011 05:00:00 +0100 5442155 http://www.medworm.com/index.php?rid=5442154&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22261 The objective of this retrospective medical record review was to examine recent trends in anti‐D use to treat ITP and rates of adverse events in a single large pediatric hematology program. Over a 7 year period, 176 (35%) of 502 ITP patients at our center received anti‐D. Anti‐D was the second most commonly prescribed drug for ITP from 2003‐2010 overall and was given first most frequently (41%). 64% of patients responded to anti‐D, but 36% had adverse effects, including 5 patients requiring hospitalization. From 2003 to 2010, the use of anti‐D as an initial therapy for ITP significantly decreased (p<0.001). This trend preceded the 2010 FDA black box warning. In our experience, anti‐D was associated with a significant number of adverse effects when used as a treatment for I... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442154 Wed, 23 Nov 2011 05:00:00 +0100 5442154 http://www.medworm.com/index.php?rid=5442153&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22260 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442153 Wed, 23 Nov 2011 05:00:00 +0100 5442153 http://www.medworm.com/index.php?rid=5442152&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22259 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442152 Wed, 23 Nov 2011 05:00:00 +0100 5442152 http://www.medworm.com/index.php?rid=5423194&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22257 AbstractChronic myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS) have an inherent tendency to progress to acute myeloid leukemia (AML). Using high‐resolution SNP microarrays, we studied a total of 517 MPN and MDS patients in different disease stages, including 77 AML cases with previous history of MPN (N=46) or MDS (N=31). Frequent chromosomal deletions of variable sizes were detected allowing the mapping of putative tumor suppressor genes involved in the leukemic transformation process. We detected frequent deletions on the short arm of chromosome 6 (del6p). The common deleted region on 6p mapped to a 1.1 Mbp region and contained only the JARID2 gene ‐ member of the Polycomb repressive complex 2 (PRC2). When we compared the frequency of del6p between chronic and ... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5423194 Sat, 19 Nov 2011 10:48:08 +0100 5423194 http://www.medworm.com/index.php?rid=5409630&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22210 AbstractDisease overview: Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by stem cell‐derived clonal myeloproliferation, abnormal cytokine expression, bone marrow fibrosis, anemia, splenomegaly, extramedullary hematopoiesis (EMH), constitutional symptoms, cachexia, leukemic progression, and shortened survival.Diagnosis: Diagnosis is based on bone marrow morphology. The presence of fibrosis, JAK2/MPL mutation or +9/13q‐ cytogenetic abnormality is supportive but not essential for diagnosis. Prefibrotic PMF mimics essential thrombocythemia in its presentation and the distinction is prognostically relevant. Differential diagnosis of myelofibrosis should include chronic myelogenous leukemia, myelodysplastic syndromes, chronic myelomonocytic leukemia, and acute my... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5409630 Thu, 17 Nov 2011 01:32:36 +0100 5409630 http://www.medworm.com/index.php?rid=5409627&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22256 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5409627 Thu, 17 Nov 2011 01:30:31 +0100 5409627 http://www.medworm.com/index.php?rid=5409628&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22255 AbstractThe prevention of Rhesus D alloimmunization through Rh immune globulin (RhIg) administration is the major indication for the accurate detection and quantification of fetomaternal hemorrhage (FMH). In the setting of D incompatibility, D‐positive fetal cells can sensitize the D‐negative mother, resulting in maternal anti‐D alloantibody production. These anti‐D alloantibodies may lead to undesirable sequelae such as hemolytic disease of the newborn (HDN). Since the widespread adoption of FMH screening and RhIg immunoprophylaxis, the overall risk of Rh alloimmunization and infant mortality from HDN has substantially decreased. The rosette screen, the initial test of choice, is highly sensitive in qualitatively detecting 10 mL of fetal whole blood in the maternal circulation. As... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5409628 Wed, 16 Nov 2011 05:00:00 +0100 5409628 http://www.medworm.com/index.php?rid=5545205&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22252 AbstractRecently, nontumor specific circulating DNA was shown to be elevated in a broad range of lymphomas, implicating a role as a potential biomarker. Epstein‐Barr virus' (EBV) presence within a proportion of lymphomas implies EBV‐DNA has potential as a lymphoma‐specific disease response biomarker. However, application would be restricted to EBV‐associated lymphomas. Neither detailed comparison has been performed of lymphoma‐specific versus nonspecific DNA as disease response biomarkers nor have the kinetics of circulating DNA during treatment been established, and the optimal methodology remains unknown. We prospectively evaluated DNA levels and clinical response of 63 lymphoma patients. DNA was measured in paired serum, plasma, and cell samples at five predetermined time‐po... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5545205 Tue, 08 Nov 2011 05:00:00 +0100 5545205 http://www.medworm.com/index.php?rid=5388875&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22253 AbstractLeukocyte adhesion deficiencies are rare clinical syndromes of impaired host defense that provide novel insights into regulation of immune and inflammatory responses (1,2). Leukocyte adhesion deficiency (LAD)‐I variant (LAD‐Iv), also called LAD‐III, is a unique disorder in which inside‐out signaling of β1, β2, and β3 integrins on leukocytes and platelets is disrupted, leading to impaired cellular adhesion, recurrent infections, and bleeding [1‐3]. We originally reported the second patient with this disorder to be identified and characterized the adhesive deficiencies and functional phenotype of this subject's leukocytes [4]. Here we show that the molecular defect in this index subject is a new mutation in FERMT3 (KINDLIN‐3) which encodes KINDLIN‐3, a cytoskeletal p... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388875 Tue, 08 Nov 2011 05:00:00 +0100 5388875 http://www.medworm.com/index.php?rid=5388874&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22252 AbstractRecently, non‐tumor specific circulating DNA was shown to be elevated in a broad‐range of lymphomas, implicating a role as a potential biomarker. Epstein‐Barr virus' (EBV) presence within a proportion of lymphomas implies EBV‐DNA has potential as a lymphoma‐specific disease response biomarker. However application would be restricted to EBV‐associated lymphomas. No detailed comparison has been performed of lymphoma‐specific versus non‐specific DNA as disease response biomarkers. Nor have the kinetics of circulating DNA during treatment been established, and the optimal methodology remains unknown. We prospectively evaluated DNA levels and clinical response of 63 lymphoma patients. DNA was measured in paired serum, plasma and cell samples at five pre‐determined time... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388874 Tue, 08 Nov 2011 05:00:00 +0100 5388874 http://www.medworm.com/index.php?rid=5388873&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22251 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388873 Tue, 08 Nov 2011 05:00:00 +0100 5388873 http://www.medworm.com/index.php?rid=5388872&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22250 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388872 Tue, 08 Nov 2011 05:00:00 +0100 5388872 http://www.medworm.com/index.php?rid=5388876&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22245 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388876 Mon, 07 Nov 2011 05:00:00 +0100 5388876 http://www.medworm.com/index.php?rid=5388877&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22247 Conclusion:Pituitary iron overload and volume loss were independently predictive of hypogonadism. Many patients with moderate‐to‐severe pituitary iron overload retained normal gland volume and function, representing a potential therapeutic window. The subset of hypogonadal patients having preserved gland volumes may also explain improvements in pituitary function observed following intensive chelation therapy. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388877 Fri, 04 Nov 2011 04:00:00 +0100 5388877 http://www.medworm.com/index.php?rid=5572817&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22244 AbstractProtease‐activated receptors (PAR)‐1 and ‐4 are the principal receptors for thrombin‐mediated platelet activation. Functional genetic variation has been described in the human PAR1 gene, but not in the PAR4 gene (F2RL3). We sought to identify variants in and around F2RL3 and to determine their association with perioperative myocardial injury (PMI) after coronary artery bypass graft surgery. We further explored possible mechanisms for F2RL3 single nucleotide polymorphism (SNP) associations with PMI including altered receptor expression and platelet activation. Twenty‐three SNPs in the F2RL3 gene region were genotyped in two phases in 934 Caucasian subjects. Platelets from 43 subjects (23 major allele, 20 risk allele) homozygous for rs773857 (SNP with the strongest associat... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5572817 Wed, 02 Nov 2011 04:00:00 +0100 5572817 http://www.medworm.com/index.php?rid=5388878&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22244 Conclusion:We conclude that rs773857 risk allele homozygotes are associated with risk for increased platelet count and hyperactivity. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388878 Wed, 02 Nov 2011 04:00:00 +0100 5388878 http://www.medworm.com/index.php?rid=5401391&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22254 AbstractD antigen is the most immunogenic and clinically relevant antigen within the complex Rh blood group system. Variability of D antigen expression was first described decades ago, but has rarely been investigated quantitatively, particularly in the context of RHD zygosity along with RhCcEe serological phenotype. With IRB approval, 107 deidentified blood samples were analyzed. Rh phenotypes were determined serologically by saline technique using monoclonal antibodies against D, C, c, E, and e antigens. RHD zygosity was determined using both PCR‐restriction fragment length polymorphisms and quantitative real‐time PCR techniques. A novel and robust method was developed for quantitation of erythrocyte D antigen sites using calibrated microspheres and flow cytometry, allowing correlati... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5401391 Tue, 01 Nov 2011 04:00:00 +0100 5401391 http://www.medworm.com/index.php?rid=5388871&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22249 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388871 Tue, 01 Nov 2011 04:00:00 +0100 5388871 http://www.medworm.com/index.php?rid=5362691&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22238 AbstractCancer‐related disseminated intravascular coagulation (DIC) is a life‐threatening condition for which no effective treatment is currently available. Protein C (PC), a modulator of coagulation as well as the inflammatory system, has been successfully tested (in its activated recombinant form [a‐rPC]) in sepsis‐related coagulopathy, but with an increased risk for major bleeding. Plasma‐derived PC (pd‐PC) is more suitable than a‐rPC in patients at high risk from bleeding due to its self‐limiting process. We carried out a single arm study evaluating the role of pd‐PC in adult cancer patients with overt DIC. Over a period of 3 years, we treated 19 patients with overt DIC and a PC plasma concentration <50%; all received PC concentrate (Ceprotin®, Baxter) for 72 hour... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5362691 Tue, 01 Nov 2011 04:00:00 +0100 5362691 http://www.medworm.com/index.php?rid=5362696&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22243 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5362696 Mon, 31 Oct 2011 04:00:00 +0100 5362696 http://www.medworm.com/index.php?rid=5362695&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22242 AbstractThe management of congenital and acquired neutropenias presents some differences according to the type of the disease. Treatment with recombinant human granulocyte‐colony stimulating factor (G‐CSF) is not standardized and scanty data are available on the best schedule to apply. The frequency and the type of longitudinal controls in patients affected with neutropenias are not usually discussed in the literature. The Neutropenia Committee of the Marrow Failure Syndrome Group (MFSG) of the Associazione Italiana di Emato‐Oncologia Pediatrica (AIEOP) elaborated this document following design and methodology formerly approved by the AIEOP board. The panel of experts reviewed the literature on the topic and participated in a conference producing a document that includes recommendati... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5362695 Mon, 31 Oct 2011 04:00:00 +0100 5362695 http://www.medworm.com/index.php?rid=5362694&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22241 AbstractAccording to World Health Organization (WHO)‐defined criteria, patients presenting clinically as Essential Thrombocythemia (ET) may show early Primary Myelofibrosis (PMF) with accompanying thrombocythemia.1 Previous clinicopathological studies revealed that laboratory parameters like gender‐matched hemoglobin (Hb), white blood cell count (WBC) and particularly lactate dehydrogenase (LDH) values are significantly different in PMF.2 By strictly applying the WHO criteria, our investigation was aimed to study sensitivity and specificity of these features in an exploratory cohort of 536 patients and to validate the results on an independently recruited series of 321 strictly corresponding patients. The discriminatory power of these parameters (Hb,WBC,LDH) was tested by plotting thei... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5362694 Mon, 31 Oct 2011 04:00:00 +0100 5362694 http://www.medworm.com/index.php?rid=5362693&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22240 AbstractIn a patient with suspected lymphoma, it is considered desirable to confirm the diagnosis by excisional biopsy of enlarged lymph nodes. However, sometimes the ideal nodes are positioned internally, requiring a deep invasive procedure for access, or the patient may have underlying medical conditions that make it risky to perform such an invasive procedure. Under a protocol approved by our institution's review board (IRB), we reviewed five patients in whom superficial lymph nodes were biopsied which were smaller than usually considered optimal for diagnosis (≤ 2 cm). In each of these cases, the biopsy yielded diagnostic information upon which treatment could be based, sparing the patient a deep invasive procedure. We suggest that in situations in which large internal lymph nodes ar... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5362693 Mon, 31 Oct 2011 04:00:00 +0100 5362693 http://www.medworm.com/index.php?rid=5362692&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22239 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5362692 Mon, 31 Oct 2011 04:00:00 +0100 5362692 http://www.medworm.com/index.php?rid=5349005&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22236 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5349005 Wed, 26 Oct 2011 21:48:11 +0100 5349005 http://www.medworm.com/index.php?rid=5337259&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22225 AbstractClinical trials in hemostasis and thrombosis (HT) are needed to guide medical practice and future research. Providing public support for trials that could have the greatest impact on clinical care has been a major challenge. The National, Heart, Lung and Blood Institute (NHLBI) convened a State of the Science meeting in Bethesda on September 14th and 15th, 2009 to identify Phase II and III clinical trials in HT that could have critical impact on healthcare. An oversight committee composed of representatives of the NHLBI and three experienced extramural investigators chose chairs of subcommittees representing six broad areas of investigation in adult and pediatric HT. Chairs were charged with identifying important, feasible proposals. Nineteen trial concepts were presented at this p... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5337259 Sat, 22 Oct 2011 23:21:35 +0100 5337259 http://www.medworm.com/index.php?rid=5442158&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22235 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442158 Sat, 22 Oct 2011 04:00:00 +0100 5442158 http://www.medworm.com/index.php?rid=5409629&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22236 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5409629 Sat, 22 Oct 2011 04:00:00 +0100 5409629 http://www.medworm.com/index.php?rid=5401394&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22232 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5401394 Sat, 22 Oct 2011 04:00:00 +0100 5401394 http://www.medworm.com/index.php?rid=5401393&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22234 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5401393 Sat, 22 Oct 2011 04:00:00 +0100 5401393 http://www.medworm.com/index.php?rid=5349010&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22231 We describe a patient with Sezary syndrome who was monitored for drug‐induced immunosuppression while undergoing treatment with vorinostat,. Analysis of the patient's natural killer cell function before and after initiation of treatment confirmed inhibition of this important cell‐mediated immune function, In addition the in vitro effects of vorinostat on the immunity of healthy volunteers confirmed that this class of drug can profoundly suppress multiple arms of the cellular immune response. These findings raise concerns of increased susceptibility to infection in this high‐risk population. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5349010 Sat, 22 Oct 2011 04:00:00 +0100 5349010 http://www.medworm.com/index.php?rid=5349009&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22232 AbstractDarinaparsin is a novel organic arsenic compound that is being developed to improve the efficacy and therapeutic index of arsenic as an antineoplastic agent. It has activity in preclinical models of hematological malignancies and we set out to test it in patients with refractory lymphoma. In this multicenter, phase II trial, patients with relapsed or refractory Hodgkin (HL) and non‐Hodgkin lymphoma (NHL) were treated with darinaparsin 300mg/m2 intravenously daily for 5 consecutive days every 28 days, for up to 6 cycles. The primary endpoint was the overall response rate. Twenty‐nine heavily pretreated patients with lymphoma (22 with NHL and 7 with HL) were enrolled. There was one complete response (CR), 1 unconfirmed CR, 3 partial responses (PR) and 4 with stable disease (SD), ... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5349009 Sat, 22 Oct 2011 04:00:00 +0100 5349009 http://www.medworm.com/index.php?rid=5349008&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22233 AbstractNinety‐four Mayo Clinic patients with myelofibrosis (MF) participated in two consecutive clinical trials of pomalidomide (0.5‐3.5 mg/day), with or without prednisone. Overall anemia response was 27% and increased to 53% in JAK2V617F‐positive patients with <10 cm palpable splenomegaly and <5% circulating blasts; response rate was 0% in mutation‐negative patients with either =10 cm splenomegaly or =5% circulating blasts (p=0.0001). Median duration of anemia response was 16 months. Treatment effect on splenomegaly was negligible. To date, pomalidomide therapy has been discontinued in 86 (91%) patients at a rate of 68% at 1 year and 89% at 2 years. Grade 1 sensory neuropathy developed in 4 (13%) of 30 patients treated for =1 year. Risk‐adjusted survival in pomalidomide�... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5349008 Sat, 22 Oct 2011 04:00:00 +0100 5349008 http://www.medworm.com/index.php?rid=5349007&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22234 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5349007 Sat, 22 Oct 2011 04:00:00 +0100 5349007 http://www.medworm.com/index.php?rid=5349006&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22235 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5349006 Sat, 22 Oct 2011 04:00:00 +0100 5349006 http://www.medworm.com/index.php?rid=5442159&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22225 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442159 Fri, 21 Oct 2011 04:00:00 +0100 5442159 http://www.medworm.com/index.php?rid=5526255&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22226 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5526255 Thu, 20 Oct 2011 04:00:00 +0100 5526255 http://www.medworm.com/index.php?rid=5442161&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22223 AbstractPancreatic iron overload and diabetes mellitus (DM) are common in thalassemia major patients. However, the relationship between iron stores and glucose disturbances is not well defined. We used a frequently sampled oral glucose tolerance test (OGTT), coupled with mathematical modeling, and magnetic resonance imaging (MRI) to examine the impact of pancreatic, cardiac, and hepatic iron overload on glucose regulation in 59 patients with thalassemia major. According to OGTT results, 11 patients had DM, 12 had impaired glucose tolerance (IGT), 8 had isolated impaired fasting glucose (IFG), and 28 patients had normal glucose tolerance (NGT). Patients with DM had significantly impaired insulin sensitivity and insulin release. Insulin resistance was most strongly associated with markers of... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442161 Thu, 20 Oct 2011 04:00:00 +0100 5442161 http://www.medworm.com/index.php?rid=5442160&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22224 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5442160 Thu, 20 Oct 2011 04:00:00 +0100 5442160 http://www.medworm.com/index.php?rid=5337266&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22223 Conclusions:Our study indicates that pancreatic iron is the strongest predictor of beta cell toxicity, but total body iron burden, age, and body habitus also influence glucose regulation. We also demonstrate that MRI and fasting glucose/insulin are complementary screening tools, reducing the need for oral glucose tolerance testing, and identify high‐risk patients prior to irreversible pancreatic damage. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5337266 Thu, 20 Oct 2011 04:00:00 +0100 5337266 http://www.medworm.com/index.php?rid=5337265&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22224 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5337265 Thu, 20 Oct 2011 04:00:00 +0100 5337265 http://www.medworm.com/index.php?rid=5337264&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22226 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5337264 Thu, 20 Oct 2011 04:00:00 +0100 5337264 http://www.medworm.com/index.php?rid=5337263&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22227 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5337263 Thu, 20 Oct 2011 04:00:00 +0100 5337263 http://www.medworm.com/index.php?rid=5337262&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22228 This study underscores the need for better monitoring of iron burden with timely treatment adjustments in chronically transfused children with SCA. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5337262 Thu, 20 Oct 2011 04:00:00 +0100 5337262 http://www.medworm.com/index.php?rid=5337261&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22229 AbstractPatients undergoing allogeneic hematopoietic stem cell transplant (allo HCT) have a higher incidence of infections partly due to secondary hypogammaglobulinemia. We evaluated the role of IVIG in allo HCT patients who received prophylactic IVIG 200 mg/kg once weekly regardless of IgG level (Group 1, n=115) compared to patients who received IVIG based on IgG level <400 mg/dl (Group 2, n=114). Primary endpoints were the utilization of IVIG, incidence of veno‐occlusive disease (VOD), graft versus host disease (GVHD), and documented infections within the first 100 days after allo HCT. Patients in both groups were similar except for a higher number of matched unrelated donor (MUD) transplants in group 2 (62 vs 41, p=0.01). There were no significant differences in the incidence all g... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5337261 Thu, 20 Oct 2011 04:00:00 +0100 5337261 http://www.medworm.com/index.php?rid=5337260&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22230 In conclusion, the use of VADs in SCD was linked to a significant rate of infection and thrombosis. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5337260 Thu, 20 Oct 2011 04:00:00 +0100 5337260 http://www.medworm.com/index.php?rid=5316384&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22217 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316384 Sat, 15 Oct 2011 12:27:20 +0100 5316384 http://www.medworm.com/index.php?rid=5316383&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22139 AbstractDisease overview: Cutaneous T‐cell lymphomas are a heterogenous group of T‐cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis fungoides (MF) or Sézary syndrome (SS).Diagnosis: The diagnosis of MF or SS requires the integration of clinical and histopathologic data.Risk‐adapted therapy: Tumor, node, metastasis, and blood (TNMB) staging remains the most important prognostic factor in MF/SS and forms the basis for a “risk‐adapted,” multidisciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin‐directed therapies is preferred, as both disease‐specific and overall survival for these patients is favorable. In contrast, patients with advanced‐stage disease with si... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316383 Sat, 15 Oct 2011 12:27:09 +0100 5316383 http://www.medworm.com/index.php?rid=5316374&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22220 Conclusions:RaISS is a new simple and easily available scoring system that, accurately defining the risk of progression, can allow to identify patients who could deserve further treatment after transplant (consolidation, maintenance). Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316374 Sat, 15 Oct 2011 12:25:16 +0100 5316374 http://www.medworm.com/index.php?rid=5526253&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22220 In conclusion, RaISS is a new simple and easily available scoring system that, accurately defining the risk of progression, can allow to identify patients who could deserve further treatment after transplant (consolidation, maintenance). Am. J. Hematol. 2011. © 2011 Wiley Periodicals, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5526253 Fri, 14 Oct 2011 04:00:00 +0100 5526253 http://www.medworm.com/index.php?rid=5401392&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22222 AbstractAs the number of anticoagulant drugs increases and new ones are brought to market, the utility of the routine screening coagulation tests of today—namely the prothrombin time and activated partial thromboplastin time—will be significantly reduced in many clinical situations. Although the new anticoagulants are designed to require less frequent monitoring, it is imperative that the proper test is selected in situations where monitoring is needed. In addition, tests that are designed for the new generation of drugs may be informative in certain situations for monitoring the anticoagulants that have been in use for many years. Here, we present the chromogenic antifactor Xa assay and demonstrate its utility and its limitations in monitoring three anticoagulant drugs (unfractionated... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5401392 Fri, 14 Oct 2011 04:00:00 +0100 5401392 http://www.medworm.com/index.php?rid=5316376&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22222 AbstractAs the number of anticoagulant drugs increases and new ones are brought to market, the utility of the routine screening coagulation tests of today – namely the prothrombin time and activated partial thromboplastin time – will be significantly reduced in many clinical situations. Although the new anticoagulants are designed to require less frequent monitoring, it is imperative that the proper test is selected in situations where monitoring is needed. In addition, tests that are designed for the new generation of drugs may be informative in certain situations for monitoring the anticoagulants that have been in use for many years. Here we present the chromogenic anti‐factor Xa assay, and demonstrate its utility and its limitations in monitoring three anticoagulant drugs (unfract... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316376 Fri, 14 Oct 2011 04:00:00 +0100 5316376 http://www.medworm.com/index.php?rid=5316375&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22221 AbstractFetal hemoglobin (HbF) is a major modifier of disease severity in sickle cell anemia (SCA). Three major HbF quantitative trait loci (QTL) are known: the Xmn I site upstream of Gγ‐ globin gene (HBG2) on chromosome 11p15, BCL11A on chromosome 2p16, and HBS1L‐MYB intergenic polymorphism (HMIP) on chromosome 6q23. However, the roles of these QTLs in SCA patients with uncharacteristically high HbF are not known. We studied 20 African American SCA patients with markedly elevated HbF (mean 17.2%). They had significantly higher minor allele frequencies (MAF) in two HbF QTLs, BCL11A and HMIP, compared with those with low HbF. A 3‐bp (TAC) deletion in complete linkage disequilibrium (LD) with the minor allele of rs9399137 in HMIP was also present significantly more often in these pati... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316375 Fri, 14 Oct 2011 04:00:00 +0100 5316375 http://www.medworm.com/index.php?rid=5469824&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22218 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5469824 Tue, 11 Oct 2011 04:00:00 +0100 5469824 http://www.medworm.com/index.php?rid=5401396&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22212 AbstractMyeloproliferative neoplasms (MPNs) are characterized by overproduction of mature functional blood cells and are often associated with an acquired genetic mutation of Janus Kinase 2V617F. The etiology of MPNs remains unknown. The aim of this article was to review and collate all known published data investigating environmental and lifestyle factors associated with MPNs. Medline, Embase, PubMed, Cochrane, and Web of Science were systematically searched using terms for MPNs and observational study designs to identify studies investigating the risk factors for MPNs published before March 2010. Of 9,156 articles identified, 19 met the selection criteria. Although the studies exhibited heterogeneity, in case definitions, study design, and risk factors investigated, several themes emerge... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5401396 Tue, 11 Oct 2011 04:00:00 +0100 5401396 http://www.medworm.com/index.php?rid=5316380&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22218 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316380 Tue, 11 Oct 2011 04:00:00 +0100 5316380 http://www.medworm.com/index.php?rid=5316379&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22212 Conclusions:The vast heterogeneity in studies identified as part of this review suggests that large scale systematic assessment of aetiological factors associated with MPNs is warranted. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316379 Tue, 11 Oct 2011 04:00:00 +0100 5316379 http://www.medworm.com/index.php?rid=5316378&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22213 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316378 Tue, 11 Oct 2011 04:00:00 +0100 5316378 http://www.medworm.com/index.php?rid=5316377&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22214 AbstractDespite the increased use of intensive immunosuppressive chemo‐immunotherapies in patients with lymphoma observed in the last decade, current data on Cytomegalovirus (CMV) infection following autologous stem cell transplantation (Auto‐SCT) are very limited. To address this peculiar aspect, a retrospective study on a cohort of 128 adult patients consecutively transplanted for lymphoma in 3 Hematology Institutions was performed with the aim to determine the incidence of and the risk factors for CMV symptomatic infection and/or end‐organ disease. Sixteen patients (12.5%) required specific antiviral therapy and 4/16 died (25%); transplant‐related mortality (TRM) was significantly influenced by CMV infection (P=0.005). In univariate analysis, a pre‐transplant HBcIgG seropositi... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316377 Tue, 11 Oct 2011 04:00:00 +0100 5316377 http://www.medworm.com/index.php?rid=5316382&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22216 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316382 Mon, 10 Oct 2011 04:00:00 +0100 5316382 http://www.medworm.com/index.php?rid=5316381&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22215 AbstractRecent clinical trials with JAK or mTOR inhibitors in primary myelofibrosis (PMF) have identified pruritus as one of the most treatment‐responsive disease traits. However, little is known about the prevalence of pruritus in PMF or its clinical and laboratory correlates. Among 566 consecutive patients with PMF seen at our institution, the presence or absence of pruritus was documented in 90 (16%) and 146 (26%) patients, respectively. Patients with pruritus were less likely to express MPLW515 (0% vs. 10%; p=0.02) or leukopenia (8% vs. 24%; p=0.002). The latter association was more pronounced in the absence of JAK2 or MPL mutations. Pruritus also clustered with marked leukocytosis (23% vs. 11%; p=0.01) and JAK2V617F (71% vs. 59%; p=0.08). Pruritus did not correlate with karyotype (p... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5316381 Mon, 10 Oct 2011 04:00:00 +0100 5316381 http://www.medworm.com/index.php?rid=5286678&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22211 AbstractImmune thrombocytopenia (ITP) is characterized by platelet clearance mediated primarily by autoantibodies against the platelet GPIIbIIIa and/or GPIbα. Steroid therapy is a first‐line treatment for ITP. However, some patients are refractory to this therapy and currently no method can predict which patients will respond. To evaluate whether steroids are equally efficacious in treating patients with ITP caused by anti‐GPIIbIIIa versus anti‐GPIbα antibodies, we performed a retrospective study on 176 newly diagnosed acute ITP patients who had severe bleeding symptoms and were admitted as resident patients to the hospital. The patients were treated first with intravenous administration of high‐dose Dexamethasone, followed by oral administration of Prednisone. Response to therap... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5286678 Sat, 01 Oct 2011 04:00:00 +0100 5286678 http://www.medworm.com/index.php?rid=5274852&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22205 Conclusions:Bradycardia and repolarization abnormalities on 12‐lead electrocardiography were the most specific markers for cardiac iron in thalassemia major. Changes in these variables may be helpful to stratify cardiac risk when cardiac MRI is unavailable. However, diagnostic algorithms need to be vetted on larger and more diverse patient populations and longitudinal studies are necessary to determine reversibility of the observed abnormalities. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5274852 Sat, 01 Oct 2011 04:00:00 +0100 5274852 http://www.medworm.com/index.php?rid=5274857&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22209 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5274857 Fri, 30 Sep 2011 04:00:00 +0100 5274857 http://www.medworm.com/index.php?rid=5274856&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22208 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5274856 Fri, 30 Sep 2011 04:00:00 +0100 5274856 http://www.medworm.com/index.php?rid=5274855&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22207 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5274855 Fri, 30 Sep 2011 04:00:00 +0100 5274855 http://www.medworm.com/index.php?rid=5274853&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22206 ConclusionThe global haemostatic potential is increased and reflects the hypercoagulable state of SCD patients even at steady‐state. The relevance of this finding with respect to the risk of thrombotic complications of the disease needs further investigation. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc. (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5274853 Fri, 30 Sep 2011 04:00:00 +0100 5274853 http://www.medworm.com/index.php?rid=5261860&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22204 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5261860 Thu, 29 Sep 2011 10:29:50 +0100 5261860 http://www.medworm.com/index.php?rid=5401395&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22202 This study was conducted to further investigate the relationship between AKI and ES. Data were collected from 377 AL patients who underwent ASCT from 7/1997 to 10/2009. Patients who experienced an elevation of serum creatinine >0.5 mg/dL within 4 days of leukocyte engraftment and anyone who presented with signs associated with ES regardless of renal manifestations were included. Forty‐one patients met criteria. Twelve were excluded for positive cultures (10), acute interstitial nephritis (1), and acute cellular rejection (1). In addition to AKI (93.1%), patients also exhibit fever (82.7%), hypotension (51.7%), rash (48.2%), edema (93.1%), diarrhea (69.0%), conjunctival hemorrhage (31.0%), pulmonary edema (31.0%), pulmonary hemorrhage (13.8%), and transient encephalopathy (17.2%). Pati... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5401395 Mon, 26 Sep 2011 04:00:00 +0100 5401395 http://www.medworm.com/index.php?rid=5388880&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22199 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388880 Mon, 26 Sep 2011 04:00:00 +0100 5388880 http://www.medworm.com/index.php?rid=5388879&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22201 AbstractThis Phase 1/2 study aimed to determine optimal doses of daunorubicin (DNR; mg/m2) and cytarabine (mg/m2) to be combined with fractionated doses of gemtuzumab ozogamicin (GO, Mylotarg®; 3 mg/m2 on day 1, 4, and 7) satisfying safety requirements. Three dose levels of DNR/AraC were investigated namely (45, 100), (60, 100), and (60, 200). Patients included were acute myeloid leukemia in first relapse, aged 50–70 years. Hematological recovery was 31 days for neutrophil and 32 days for platelet counts. A documented infectious episode > Grade 2 occurred in 11/20 patients (55%). None of the 20 patients had signs of veno‐occlusive disease. Overall, eleven patients reached complete remission (CR), two CR with incomplete platelets recovery. The results showed that combination of frac... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5388879 Mon, 26 Sep 2011 04:00:00 +0100 5388879 http://www.medworm.com/index.php?rid=5261866&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22198 The objective of this study was to determine the clinical efficacy, safety and tolerability of panobinostat in this patient population. The study would stop early if there was a chance greater than 99% that the average overall response rate was less than 15%. Thirteen patients were treated: Median age was 70 years (range 47 to 84), 70% were transfusion dependent, 70% had intermediate‐1 risk MDS. Most patients were diploid but one patient with del(5q), one with trisomy 8, one with complex cytogenetics and 2 with deletion of 20q were included. Approximately 40% had previous therapy for MDS. Of the 13 patients, 1(8%) achieved hematological improvement (HI‐E and HI‐P, duration 3 months) and 6 (46%) had stable disease for a median duration of 6 months (range 1.7‐13.6). Treatment was wit... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5261866 Mon, 26 Sep 2011 04:00:00 +0100 5261866 http://www.medworm.com/index.php?rid=5261865&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22199 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5261865 Mon, 26 Sep 2011 04:00:00 +0100 5261865 http://www.medworm.com/index.php?rid=5261864&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22200 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5261864 Mon, 26 Sep 2011 04:00:00 +0100 5261864 http://www.medworm.com/index.php?rid=5261863&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22201 AbstractThis Phase 1/2 study aimed to determine optimal doses of daunorubicin (mg/m2) and cytarabine (mg/m2) to be combined with fractionated doses of gemtuzumab ozogamicin (GO, Mylotarg®; 3 mg/m2 on day 1, 4, and 7) satisfying safety requirements. Three dose levels of DNR/AraC were investigated namely (45, 100), (60, 100), and (60, 200). Patients included were AML in first relapse, aged 50 to 70 years. Hematological recovery was 31 days for neutrophil and 32 days for platelet counts. A documented infectious episode > grade 2 occurred in 11/20 patients (55%). None of the 20 patients had signs of veno occlusive disease. Overall, eleven patients reached complete remission (CR), two CR with incomplete platelets recovery (CRp).The results showed that combination of fractionated GO doses wi... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5261863 Mon, 26 Sep 2011 04:00:00 +0100 5261863 http://www.medworm.com/index.php?rid=5261862&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22202 This study was conducted to further investigate the relationship between AKI and ES.Data was collected from 377 AL patients who underwent ASCT from 7/1997 to 10/2009. Patients who experienced an elevation of serum creatinine > 0.5 mg/dl within 4 days of leukocyte engraftment and anyone who presented with signs associated with ES regardless of renal manifestations were included.Forty‐one patients met criteria. Twelve were excluded for positive cultures (10), acute interstitial nephritis (1) and acute cellular rejection (1). In addition to AKI (93.1%), patients also exhibit fever (82.7%), hypotension (51.7%), rash (48.2%), edema (93.1%), diarrhea (69.0%), conjunctival hemorrhage (31.0%), pulmonary edema (31.0%), pulmonary hemorrhage (13.8%) and transient encephalopathy (17.2%). Patient ... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5261862 Mon, 26 Sep 2011 04:00:00 +0100 5261862 http://www.medworm.com/index.php?rid=5261861&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22203 AbstractNon‐transferrin bound iron (NTBI) is commonly detected in patients with systemic iron overload whose serum iron‐binding capacity has been surpassed. It has been perceived as an indicator of iron overload, impending organ damage and a chelation target in poly‐transfused thalassemia patients. However, NTBI is a heterogeneous entity comprising various iron complexes, including a significant redox‐active and readily chelatable fraction, which we have designated 'labile plasma iron' (LPI). We found that LPI levels can be affected by plasma components such as citrate, uric acid and albumin. However, the inclusion of a mild metal mobilizing agent in the LPI assay (designated here as 'eLPI'), at concentrations that do not affect transferrin‐bound iron, largely overcomes such effe... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5261861 Mon, 26 Sep 2011 04:00:00 +0100 5261861 http://www.medworm.com/index.php?rid=5251565&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22194 AbstractThe genetic variants predisposing to familial myeloproliferative neoplasms (MPN) are still to be defined. Germline TP53 mutations are involved in genetic predisposition to cancer. Inactivation of the p53 pathway may occur by up regulation of the MDM2 protein. A single nucleotide polymorphisms (SNP 309 T/G) in the MDM2 promoter was shown to increase the affinity of the transcriptional factor Sp1, resulting in higher levels of MDM2 RNA and protein and subsequent attenuation of the p53 pathway. We investigated the distribution of MDM2 SNP 309 in 71 familial MPN to evaluate its influence on disease susceptibility and age of disease onset, the association with the JAK2 (V617F) mutation and the impact on disease progression. The distribution of the high risk MDM2 SNP 309 (G allele) did n... American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5251565 Sun, 25 Sep 2011 22:27:47 +0100 5251565 http://www.medworm.com/index.php?rid=5362697&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22194 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5362697 Fri, 23 Sep 2011 04:00:00 +0100 5362697 http://www.medworm.com/index.php?rid=5251566&cid=s_33582_19_f&fid=33582&url=http%3A%2F%2Fdx.doi.org%2F10.1002%252Fajh.22195 (Source: American Journal of Hematology) American Journal of Hematology journals http://www.medworm.com/rss/comments.php?id=5251566 Fri, 23 Sep 2011 04:00:00 +0100 5251566