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Investigation of the differences between the Tibetan and Han populations in the hemoglobin-oxygen affinity of red blood cells and in the adaptation to high-altitude environments.
CONCLUSIONS: The change of P50 could be a feature of the adaptation to the plateau and to avoid altitude sickness, such as high-altitude polycythemia and dyspnea. PMID: 29130390 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - November 14, 2017 Category: Hematology Tags: Hematology Source Type: research

Corrigendum.
Authors: PMID: 29130398 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - November 14, 2017 Category: Hematology Tags: Hematology Source Type: research

Corrigendum.
Authors: PMID: 29115182 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - November 10, 2017 Category: Hematology Tags: Hematology Source Type: research

Identification of a de novo ANK1 mutation in a Chinese family with hereditary spherocytosis.
DISCUSSION: Our finding suggested that a de novo nonsense mutation in ANK1 may be causative to HS which plays an important role in supplementing the mutational spectrum of the ANK1 and explaining the mechanism of HS. Our study also indicated that WES can be an effective and accurate diagnostic tool in the discovery of causative mutations in genetic heterogeneous Mendelian disorders. PMID: 29099659 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - November 4, 2017 Category: Hematology Tags: Hematology Source Type: research

Gestational age and childhood leukemia: A meta-analysis of epidemiologic studies.
CONCLUSION: Our results suggest that both preterm and postterm infants have an elevated risk of developing AML. In addition, postterm birth increased the risk of childhood leukemia and ALL in cohort studies. However, more studies are warranted to validate these results and explore the biologic mechanisms underlying these relationships. PMID: 29099685 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - November 4, 2017 Category: Hematology Tags: Hematology Source Type: research

Involvement of oncogenic tyrosine kinase NPM-ALK in trifluoperazine-induced cell cycle arrest and apoptosis in ALK(+) anaplastic large cell lymphoma.
CONCLUSIONS: Our results demonstrate for the first time that TFP is capable of inducing degradation and inhibition of kinase activity of NPM-ALK, as well as the increased level of phospho-NPM-ALK, leading to the inhibition of cell growth in ALK(+) ALCL cells. TFP may have a potential to be utilized to treat this aggressive lymphoma. PMID: 29086626 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - November 1, 2017 Category: Hematology Tags: Hematology Source Type: research

Elevated plasma asymmetric dimethylarginine levels in children with beta-thalassemia major may be an early marker for endothelial dysfunction.
In this study, we aimed to investigate circulating ADMA concentrations in children with beta-thalassemia major. METHODS: Thirty-one beta-thalassemia major children aged between 4 and 16 year old and age, gender-matched 36 healthy controls were enrolled in the study. Plasma ADMA was measured along with the soluble vascular cell adhesion molecule-1 (sVCAM-1), soluble intercellular adhesion molecule-1 (sICAM-1), P-selectin, and Pentraxin-3. RESULTS: Age, gender and body mass index were similar in two groups. Plasma ADMA, sVCAM-1, and sICAM-1 measurements were significantly higher in beta-thalassemia major pa...
Source: Hematology - November 1, 2017 Category: Hematology Tags: Hematology Source Type: research

Anthropometric measurements in children having transfusion-dependent beta thalassemia.
Authors: Moiz B, Habib A, Sawani S, Raheem A, Hasan B, Gangwani M Abstract OBJECTIVES: The aim of this study was to determine the anthropometric measurements in transfusion-dependent β-thalassemia children in Pakistan. The secondary aim was to correlate serum ferritin with the physical growth. METHODS: We enrolled 367 children (aged 5-17 years) with transfusion-dependent beta-thalassemia major in the study. Anthropometric measurements, serum ferritin levels, and pre-transfusion hemoglobin levels were measured. Serum ferritin was correlated with the height z-score for age. RESULTS: Laboratory evaluation...
Source: Hematology - November 1, 2017 Category: Hematology Tags: Hematology Source Type: research

Impact of estimated glomerular filtration rate based on plasma cystatin C and serum creatinine levels before allogeneic hematopoietic cell transplantation.
Authors: Wada H, Kanda J, Akahoshi Y, Nakano H, Ugai T, Yamasaki R, Ishihara Y, Kawamura K, Sakamoto K, Ashizawa M, Sato M, Terasako-Saito K, Kimura SI, Kikuchi M, Nakasone H, Yamazaki R, Kako S, Tanihara A, Nishida J, Kanda Y Abstract BACKGROUND: No standard method for measuring renal function has been established in allogeneic hematopoietic cell transplantation (allo-HCT). METHODS: We retrospectively analyzed 80 patients with hematological diseases who underwent allo-HCT at our center. We assessed renal function using creatinine clearance (Ccr), estimated glomerular filtration rate (eGFR) based on creatinine ...
Source: Hematology - November 1, 2017 Category: Hematology Tags: Hematology Source Type: research

Serum ferritin is a different predictor from transfusion history for allogeneic transplantation outcome in patients with severe aplastic anemia.
Authors: Zhang X, Shi Y, Huang Y, Zhang G, He Y, Jiang E, Wei J, Yang D, Ma Q, Zhang R, Zhai W, Feng S, Han M Abstract OBJECTIVES: Severe aplastic anemia (SAA) patients receive more red blood cell (RBC) transfusions as supportive management. We aim to clarify the associations between transfusion history or pre-transplantation serum ferritin (SF) and the overall survival of allogeneic hematopoietic stem cell transplantation (allo-HSCT) among SAA patients. MATERIAL AND METHODS: We retrospectively investigated 96 SAA patients undergoing allo-HSCT, and grouped them according to pre-transplantation duration. Pre-tra...
Source: Hematology - October 21, 2017 Category: Hematology Tags: Hematology Source Type: research

Estrogen receptor beta expression and prognosis of diffuse large B cell lymphoma.
CONCLUSION: ERβ1 protein expression represented opposite prognostic factors in nodal vs. extra-nodal DLBCL. PMID: 29032728 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - October 18, 2017 Category: Hematology Tags: Hematology Source Type: research

Tolerability and safety of the intravenous immunoglobulin octagam( ®) 10% in patients with immune thrombocytopenia: a post-authorisation safety analysis of two non-interventional phase IV trials.
CONCLUSIONS: Routine clinical use of octagam(®) 10% was safe and well tolerated, with no unexpected safety issues, in patients with ITP. The two studies from which data were taken are registered with the International Standard Randomised Controlled Trial Number Registry, numbers ISRCTN58800347 and ISRCTN02245668. PMID: 29020871 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - October 14, 2017 Category: Hematology Tags: Hematology Source Type: research

Knockdown of REG γ inhibits the proliferation and migration and promotes the apoptosis of multiple myeloma cells by downregulating NF-κB signal pathway.
This study aimed to evaluate the effects of REGγ knockdown on the proliferation, apoptosis and migration of multiple myeloma (MM) cells, and reveal the potential regulatory mechanisms. METHODS: The expression of REGγ on myeloma cells of 28 MM patients was detected by Western blot. shRNA-REGγ-1 and shRNA-REGγ-2 were constructed to downregulate REGγ in RPMI-8226 cells. The proliferation, apoptosis and migration of transfected cells were analyzed by Cell Counting Kit 8 (CCK8), flow cytometry and transwell chamber, respectively. The expression of phosphorylated p65 (p-p65), p65, NF-kappa-B inh...
Source: Hematology - October 14, 2017 Category: Hematology Tags: Hematology Source Type: research

The potential association of tumor necrosis factor- βeta (252 G/A) cytokine gene polymorphism with immune thrombocytopenic purpura among Egyptian children.
CONCLUSION: The risk of developing ITP was not related to the studied polymorphism. PMID: 29020887 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - October 14, 2017 Category: Hematology Tags: Hematology Source Type: research

Recommendations on the use of ruxolitinib for the treatment of myelofibrosis.
CONCLUSION: With the aim to optimize the treatment of MF patients, the Belgian regulation for ruxolitinib should be revised in terms of reimbursement criteria, dose titration, stopping rules, and patient follow-up. PMID: 29022420 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - October 14, 2017 Category: Hematology Tags: Hematology Source Type: research

CALR, JAK2 and MPL mutation status in Argentinean patients with BCR-ABL1- negative myeloproliferative neoplasms.
CONCLUSION: Our study underscores the importance of JAK2, MPL and CALR genotyping for accurate diagnosis of patients with BCR-ABL1-negative MPN. PMID: 28990497 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - October 12, 2017 Category: Hematology Tags: Hematology Source Type: research

Third-line treatment with second-generation tyrosine kinase inhibitors (dasatinib or nilotinib) in patients with chronic myeloid leukemia after two prior TKIs: real-life data on a single center experience along with the review of the literature.
In this study, CML patients who received dasatinib or nilotinib as a third-line treatment were retrospectively evaluated. RESULTS: Out of 209 patients, third-line dasatinib/nilotinib was administered in 21. During the follow-up, 16 out of 21 patients gained and/or maintained an optimal response, and 4 patients died due to progression. Seventeen patients were alive at the time of the analysis, of which 13 were still on TKI, whereas 4 patients quit treatment. DISCUSSION: In patients failing two lines of TKI, dasatinib or nilotinib can be beneficial and safely administered as a third-line treatment especially in natio...
Source: Hematology - October 12, 2017 Category: Hematology Tags: Hematology Source Type: research

EgoNet identifies differential ego-modules and pathways related to prednisolone resistance in childhood acute lymphoblastic leukemia.
Authors: Jiang J, Yin XY, Song XW, Xie D, Xu HJ, Yang J, Sun LR Abstract PURPOSE: To extract feature ego-modules and pathways in childhood acute lymphoblastic leukemia (ALL) resistant to prednisolone treatment, and further to explore the mechanisms behind prednisolone resistance. MATERIALS AND METHODS: EgoNet algorithm was used to identify candidate ego-network modules, mainly via constructing differential co-expression network (DCN); selecting ego genes; collecting ego-network modules; refining candidate modules. Afterwards, statistical significance was calculated for these candidate modules. Biological functi...
Source: Hematology - October 12, 2017 Category: Hematology Tags: Hematology Source Type: research

Multivariate analysis of prognosis for patients with natural killer/T cell lymphoma-associated hemophagocytic lymphohistiocytosis.
CONCLUSION: NK/T-LAHS is a disease of poor prognosis and high mortality. NK/T-LAHS patients who achieved OR remission after the initial induction therapy had a better prognosis than non-remission patients and Allo-HSCT was an effective way to prolong the survival of NK/T-LAHS patients. However, EBV positivity in peripheral blood was a poor prognostic factor in NK/T-LAHS patients. PMID: 28982299 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - October 8, 2017 Category: Hematology Tags: Hematology Source Type: research

FC gamma receptor polymorphisms in patients with immune thrombocytopenia.
CONCLUSION: Our results suggest possible role of FCGR3A polymorphism in the etiology, development and clinical outcome of ITP, but larger prospective studies are needed to confirm these results. PMID: 28942727 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - September 27, 2017 Category: Hematology Tags: Hematology Source Type: research

Novel interactions of two α-Hb variants with SEA deletion α(0)-thalassemia: hematological and molecular analyses.
CONCLUSIONS: Identification of the patients confirmed that interaction of these rare Hb variants with α(0)-thalassemia does not lead to the Hb H disease. Differentiation of these two Hb variants from other clinically relevant hemoglobinopathies in a routine setting is, however, necessary. This can be accomplished using a combined Hb-HPLC and CE analysis followed by PCR-RFLP assays. PMID: 28945175 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - September 27, 2017 Category: Hematology Tags: Hematology Source Type: research

Innovative PCR without DNA extraction for African sickle cell disease diagnosis.
In this study, we tested the performance of a new molecular diagnostic tests on qualified samples. METHODS: The Human Hb S/C Lamp assay is a new polymerase chain reaction test able to detect HbS, HbC and HbA alleles without DNA extraction, directly on fresh or frozen blood samples, or on dried blood spots (DBS). In this study, we compared the genotyping of 248 blood samples (56 whole blood and 192 DBS) with this LAMP assay to the routine diagnostic methods performed in the genetics lab at the university hospital of Liège. RESULTS: Our results show that the LAMP method can detect HbS and HbC with an accuracy ...
Source: Hematology - September 21, 2017 Category: Hematology Tags: Hematology Source Type: research

Flow cytometric analysis of patients with hereditary spherocytosis - an Indian scenario.
This study was conducted to evaluate the utility of FC-OFT in all newly diagnosed cases of HS, to compare its diagnostic value with conventional OFT and to correlate with clinical disease severity. METHODS: In this study, the percentage of residual red cells (%RRC) was measured using flow cytometer after creating a red cell suspension. Subsequently, this was spiked with deionized water for FC-OFT in all cases of HS (n = 40), healthy subjects (n = 40) and beta-thalassemia traits (BTT) (n = 20). RESULTS: The receiver operator curve analysis defined the optimal cut-offs for FC...
Source: Hematology - September 17, 2017 Category: Hematology Tags: Hematology Source Type: research

Associations between the HLA-A/B/DRB1 polymorphisms and aplastic anemia: evidence from 17 case-control studies.
CONCLUSION: In conclusion, HLA-A/B/DRB1 polymorphisms may play an important role in AA, but higher quality and larger sample studies are needed to confirm. PMID: 28902578 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - September 15, 2017 Category: Hematology Tags: Hematology Source Type: research

High absolute basophil count is a powerful independent predictor of inferior overall survival in patients with primary myelofibrosis.
Authors: Lucijanic M, Livun A, Stoos-Veic T, Pejsa V, Jaksic O, Cicic D, Lucijanic J, Romic Z, Orehovec B, Aralica G, Miletic M, Kusec R Abstract OBJECTIVES: To investigate the clinical and prognostic significance of absolute basophil count (ABC) in patients with primary myelofibrosis (PMF). METHODS: We retrospectively investigated 58 patients with PMF treated in our institution in the period from 2006 to 2017. ABC was obtained in addition to other hematological and clinical parameters. Patients were separated into high and low ABC groups using the Receiver operating characteristic curve analysis. RESULTS: ...
Source: Hematology - September 15, 2017 Category: Hematology Tags: Hematology Source Type: research

Sex chromosome changes in leukemia: cytogenetics and molecular aspects.
CONCLUSION: The presence of these abnormalities can cause genetic instability in BM and result in the development of a malignant clone and progression of the disease. In addition, the evaluation of SCL together with the genes involved in these chromosomes can contribute to predict the disease prognosis as well as monitoring of malignancy. PMID: 28889783 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - September 12, 2017 Category: Hematology Tags: Hematology Source Type: research

The impact of FLT3 mutations on treatment response and survival in Chinese de  novo AML patients.
This study was aimed to analyze the impacts of these mutations on clinical outcomes, and assess the efficacy of different therapeutic regimens (allo-HSCT, sorafenib, or conventional chemotherapy) for AML patients with FLT3 mutations after the standard induction therapy. MATERIALS AND METHODS: We analyzed DNA samples from 158 consecutive de novo AML patients (18-60 years, excluding APL) with FLT3 mutations between July 2010 and October 2015. RESULTS: We found that AML patients with FLT3-TKD mutations have more favorable clinical outcomes than those with FLT3-ITD mutations. We also found that allo-HSCT therapy subgro...
Source: Hematology - September 8, 2017 Category: Hematology Tags: Hematology Source Type: research

Decreased levels of Th17 cells are associated with invasion fungal infections after allogeneic hematopoietic stem cell transplantation.
CONCLUSIONS: These findings supported that Th17 cells may be involved in the immune pathology of IFIs after allo-HSCT. PMID: 28880742 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - September 8, 2017 Category: Hematology Tags: Hematology Source Type: research

The impact of Fc gamma receptor IIa and IIIa gene polymorphisms on the therapeutic response of rituximab in Egyptian adult immune thrombocytopenic purpura.
CONCLUSION: The higher platelet count achieved early is predictive for a better response to rituximab later. FCγRIIA polymorphisms did not significantly influence response to rituximab in ITP. PMID: 28856973 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - September 1, 2017 Category: Hematology Tags: Hematology Source Type: research

Challenges in the management of sickle cell disease during pregnancy in Senegal, West Africa.
Authors: Faye BF, Kouame KB, Seck M, Diouf AA, Gadji M, Dieng N, Touré SA, Sall A, Toure AO, Diop S Abstract OBJECTIVES: The aim of this study was to evaluate the maternal and fetal complications in pregnant patients with sickle cell disease (SCD) and find risk factors of stillbirth. METHOD: We conducted a prospective study in pregnant women with SCD. Demographic characteristics, maternal and fetal morbi-mortality, and outcome of pregnancies were described. Risk factors of fetal loss were evaluated by comparing the parameters of the pregnancies that led to a live birth with those interrupted. RESULTS...
Source: Hematology - August 29, 2017 Category: Hematology Tags: Hematology Source Type: research

Human platelet antigen 1, 2 and 5 gene polymorphisms in Egyptians and their potential association with susceptibility to immune thrombocytopenic purpura in Egyptian patients.
CONCLUSION: HPA 2b are 2.37 times more likely to develop ITP compared to those without this allele. The relatively high allele frequency of the HPA-1b in the Egyptian population suggests that this ethnic group has a higher risk of alloimmunization. PMID: 28823219 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - August 22, 2017 Category: Hematology Tags: Hematology Source Type: research

Can biomarkers of coagulation, platelet activation, and inflammation predict mortality in patients with hematological malignancies?
This study is a prospective observational cohort study; Hypercoagulability and inflammatory biomarkers including:(1) Coagulation and fibrinolysis activation Markers (D-dimer, Fibrinogen, Antithrombin, plasminogen activator inhibitor 1 [PAI-1]);(2) Endothelium and platelet activation Markers (von Willebrand Factor [vWF], soluble P-selectin); and (3) Inflammation Markers (Tumor necrosis factor alpha [TNF-α], Interleukin-6 [IL-6]) were assayed on a group of 171 patients with hematological malignancies at time of diagnosis. They have been followed up for an average period of 416.8 days with an endpoint of mortality. ...
Source: Hematology - August 22, 2017 Category: Hematology Tags: Hematology Source Type: research

Double vs. single cord blood transplantation in adolescent and adult hematological malignancies with heavier body weight ( ≥50 kg).
CONCLUSIONS: For adolescent and adult hematological malignancies with heavier body weight (≥50kg), double-unit CBT has an inferior clinical outcome when compared with single-unit CBT having a sufficient cell dose. Double-unit CBT should only reserve for patients who need an urgent transplant but lacking of a related or unrelated donor and without an adequately dosed single CB. PMID: 28795658 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - August 12, 2017 Category: Hematology Tags: Hematology Source Type: research

Interaction between Hb E and Hb Yala (HBB:c.129delT); a novel frameshift beta globin gene mutation, resulting in Hemoglobin E/ β(0) thalassemia.
CONCLUSION: This report has provided evidence that genotype-phenotype correlation in thalassemia syndromes is highly complex and a correct clinical severity classification of thalassemia should be mainly based on clinical evaluation. PMID: 28768465 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - August 6, 2017 Category: Hematology Tags: Hematology Source Type: research

Immunophenotypic analysis of adult patients with T-cell lymphoblastic lymphoma treated with hyper-CVAD.
CONCLUSIONS: Assessment of T-cell differentiation stages in malignant T lymphoblasts would be important in choosing treatment strategies for adult patients with T-LBL. PMID: 28778131 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - August 6, 2017 Category: Hematology Tags: Hematology Source Type: research

Stigma and illness uncertainty: adding to the burden of sickle cell disease.
CONCLUSION: The study highlights socioeconomic factors to be significant to the stigma and illness uncertainty experiences in SCD. Efforts by healthcare workers to reduce patient illness uncertainty may have additional impact, reducing their stigma. PMID: 28766464 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - August 3, 2017 Category: Hematology Tags: Hematology Source Type: research

Epidemiologic study of major complications in adolescent and adult patients with thalassemia in Northeastern Thailand: the E-SAAN study phase I.
CONCLUSION: Nearly half of the patients in this cohort had disease-related complications. Splenectomy and advanced age were important factors for complication involvement. Early screening for the complications may reduce the morbidity and mortality in patients with thalassemia. PMID: 28759343 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - August 2, 2017 Category: Hematology Tags: Hematology Source Type: research

Congenital prothrombin defects: they are not only associated with bleeding but also with thrombosis: a new classification is needed.
CONCLUSIONS: These observations have required a reclassification of prothrombin defects. To the Type I and Type II defects, a Type III has to be added characterized by the absence of bleeding and the presence of venous thrombosis. It is not clear yet if this special variant of Type II defect is limited to the Arg596 mutations or if other residues may be involved. PMID: 28762299 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - August 2, 2017 Category: Hematology Tags: Hematology Source Type: research

Microsatellite Instability and Promoter Hypermethylation of DNA repair genes in Hematologic Malignancies: a forthcoming direction toward diagnostics.
CONCLUSION: The presence of specific microsatellite marker hyper-mutability and consistent promoter hypermethylation in leukemia or lymphoma can be considered as a part of routine diagnostic test in clinical laboratories. PMID: 28728506 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - July 23, 2017 Category: Hematology Tags: Hematology Source Type: research

Thalidomide has a significant effect in patients with thalassemia intermedia.
CONCLUSION: Thalidomide had a significant effect in patients with thalassemia intermedia. Further studies of a larger scale and more rigorous design are warranted. PMID: 28718348 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - July 20, 2017 Category: Hematology Tags: Hematology Source Type: research

Characteristics of bone marrow cell dysplasia and its effectiveness in diagnosing myelodysplastic syndrome.
CONCLUSION: Four specific dysplastic types possess higher diagnostic efficacy for the diagnosis of MDS. Though the dysplastic rate over 10% in any hematopoietic cell lineage presents a lower FPR, it is possibly considered to lower the diagnostic threshold of MDS if a specific dysplastic type with higher diagnostic efficacy presents. PMID: 28675126 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - July 6, 2017 Category: Hematology Tags: Hematology Source Type: research

Pretransplant serum FT3 levels in recipients predict early non-relapse mortality after myeloablative allogeneic haematopoietic cell transplantation from matched sibling donors.
CONCLUSION: A low FT3 level before conditioning may be a useful predictive biomarker for higher early NRM among patients undergoing myeloablative sibling transplantation. PMID: 28651453 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - June 29, 2017 Category: Hematology Tags: Hematology Source Type: research

Neurocognitive dysfunction in children with β thalassemia major: psychometric, neurophysiologic and radiologic evaluation.
CONCLUSION: β-TM is associated with neurocognitive impairment that can be assessed by psychometric, neurophysiologic and radiologic tests. The role of hemosiderosis and iron chelation therapy on cognitive functioning still need more research. ABBREVIATIONS: β-TM: beta thalassemia major; DFO: Dysferal; DFP: Deferiprone; DFX: Deferasirox; WISC: Wechsler Intelligence Scale for Children; VIQ: verbal IQ; PIQ: performance IQ; TIQ: total IQ; BVRT: Benton Visual Retention Test; WCST: Wisconsin Card Sort Test; MRS: Magnetic resonant spectroscopy; NAA/Cr ratio: N-acetylaspartate to creatine ratio. PMID: 28621205 [P...
Source: Hematology - June 19, 2017 Category: Hematology Tags: Hematology Source Type: research

Revisiting beta thalassemia intermedia: past, present, and future prospects.
CONCLUSION: TI is not a mild disease. The interplay of three hallmark pathophysiologic factors (ineffective erythropoiesis, chronic anemia, and iron overload) leads to the clinical presentations seen in TI. New treatment modalities are currently being investigated to broaden the options available for TI management. PMID: 28589785 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - June 9, 2017 Category: Hematology Tags: Hematology Source Type: research

Safety and efficacy of Ofatumumab in chronic lymphocytic leukemia: a systematic review and meta-analysis.
DISCUSSION: Our analysis showed PFS was statistically significantly improved with Ofatumumab-based treatments (including Ofatumumab alone, Ofatumumab plus chemotherapy) for CLL compared with observation or chemotherapy-based regimen groups. Ofatumumab had no statistically significant improvement on the OS of patients with CLL. The Ofatumumab-based therapy could generally decrease the risk of adverse effects except infusion-related reaction and infections. PMID: 28580841 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - June 7, 2017 Category: Hematology Tags: Hematology Source Type: research

Relapse of childhood acute lymphoblastic leukemia and outcomes at a reference center in Latin America: organomegaly at diagnosis is a significant clinical predictor.
CONCLUSION: A high rate of very early, CNS, and BM relapse with a considerably low 5-year OS requiring reassessment of therapy was documented. Organomegaly at diagnosis was a highly significant clinical predictor for relapse. PMID: 28580844 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - June 7, 2017 Category: Hematology Tags: Hematology Source Type: research

Interferon- γ mediates the immunosuppression of bone marrow mesenchymal stem cells on T-lymphocytes in vitro.
CONCLUSIONS: Human MSCs constitutively expressed immunosuppressive levels of PGE2, HGF and TGF-β1. The proinflammatory cytokine IFN-γ exhibited synergistic effects with MSCs on immunosuppression, possibly by up-regulating PGE2, HGF and TGF-β1 in MSCs and inducting MSCs expression of IDO, involved in tryptophan catabolism. PMID: 28581352 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - June 7, 2017 Category: Hematology Tags: Hematology Source Type: research

Positron emission tomography computed tomography features of monomorphic epitheliotropic intestinal T-cell lymphoma.
CONCLUSION: These findings showed that in contrast to classical enteropathy-associated T-cell lymphoma, where the small bowel is the exclusive primary site (owing to its origin from coeliac disease) and distant metastases even during relapse are exceptional, MEITL might on presentation and during relapse involve any part of the gut, and metastasize to multiple extra-intestinal sites. PMID: 28581364 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - June 7, 2017 Category: Hematology Tags: Hematology Source Type: research

Monoclonal B-cell lymphocytosis in blood donors in Turkey.
CONCLUSION: Large population-based studies and standardized laboratory methods are needed to determine the potential risk factors of progression to CLL, including molecular markers and genetic profile. PMID: 28583051 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - June 7, 2017 Category: Hematology Tags: Hematology Source Type: research

The role of bone marrow microenvironment in platelet production and their implications for the treatment of thrombocytopenic diseases.
CONCLUSIONS: Further definition of the role of bone marrow microenvironment in platelet generation may deepen our understanding of the underlying mechanisms as well as provide new therapeutic targets for thrombocytopenic diseases. PMID: 28569613 [PubMed - as supplied by publisher] (Source: Hematology)
Source: Hematology - June 3, 2017 Category: Hematology Tags: Hematology Source Type: research