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International Journal of Hematology

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https://www.readbyqxmd.com/read/28631178/recent-insights-into-the-molecular-basis-of-fanconi-anemia-genes-modifiers-and-drivers
#1
REVIEW
Ronald S Cheung, Toshiyasu Taniguchi
Fanconi anemia (FA), the most common form of inherited bone marrow failure, predisposes to leukemia and solid tumors. FA is caused by the genetic disruption of a cellular pathway that repairs DNA interstrand crosslinks. The impaired function of this pathway, and the genetic instability that results, is considered the main pathogenic mechanism behind this disease. The identification of breast cancer susceptibility genes (for example, BRCA1/FANCS and BRCA2/FANCD1) as being major players in the FA pathway has led to a surge in molecular studies, resulting in the concept of the FA-BRCA pathway...
June 19, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28631177/trail-in-cd8-t-cells-from-patients-with-severe-aplastic-anemia
#2
Chunyan Liu, Mengying Zheng, Tian Zhang, Rong Fu, Huaquan Wang, Ting Wang, Weiwei Qi, Zonghong Shao
Severe aplastic anemia (SAA) is an autoimmune disease caused mainly by activated T lymphocytes. Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is a member of TNF family, which can induce apoptosis and play a significant role in the pathogenesis of many autoimmune disorders. In this study, we sought to investigate the role of TRAIL in peripheral CD8+ T cells (CTLs) from SAA patients to clarify the autoimmune mechanisms of bone marrow failure in SAA. The expression of TRAIL and TRAIL-R2 in CTLs from SAA patients and normal controls were determined by flow cytometry, real-time PCR, and western blot...
June 19, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28631176/familial-predisposition-of-myeloid-malignancies-biological-and-clinical-significance-of-recurrent-germ-line-mutations
#3
EDITORIAL
Hirotaka Matsui
No abstract text is available yet for this article.
June 19, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28623609/administration-of-direct-oral-anticoagulants-in-patients-with-myeloproliferative-neoplasms
#4
Jean-Christophe Ianotto, Marie-Anne Couturier, Hubert Galinat, Dominique Mottier, Christian Berthou, Gaëlle Guillerm, Eric Lippert, Aurélien Delluc
Direct oral anticoagulants (DOACs) have been approved to treat and prevent thrombotic events. However, they are not yet labeled for use in patients with active cancers. Myeloproliferative neoplasms (MPNs) are clonal chronic disorders with a high incidence of thrombotic events, for which low-dose aspirin (LDA) is the standard drug treatment. We analyzed efficacy and safety of DOACs prescription in patients treated for MPNs. An MPN database, the OBENE registry, was established at our institution. We collected biological and clinical data from diagnosis to last follow-up for every patient included in this study...
June 16, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28616699/flt3-itd-with-dnmt3a-r882-double-mutation-is-a-poor-prognostic-factor-in-chinese-patients-with-acute-myeloid-leukemia-after-chemotherapy-or-allogeneic-hematopoietic-stem-cell-transplantation
#5
Shanhao Tang, Hongjie Shen, Xinliang Mao, Haiping Dai, Xiaming Zhu, Shengli Xue, Zixuan Ding, Jing Lu, Depei Wu, Xiaowen Tang
To investigate clinical characteristics and outcomes of transplantation in AML patients with FLT3-ITD/DNMT3A double mutation, we retrospectively analyzed 206 Chinese patients with AML after Sanger sequencing. Our analysis showed that AML patients with FLT3-ITD and DNMT3A R882 mutations had a higher white blood cell count and a lower complete remission (CR) rate after first induction chemotherapy. All 206 patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT) in status of CR. These results indicate that AML patients with FLT3-ITD and DNMT3A R882 double mutation show a higher 2-year cumulative incidence of relapse (CIR), lower 2-year overall survival (OS) rate, and lower 2-year leukocyte-free survival (LFS) after allo-HSCT...
June 14, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28616698/costs-of-hematopoietic-stem-cell-transplantation-in-a-developing-country
#6
Monica M Rivera-Franco, Eucario Leon-Rodriguez, Haydee L Castro-Saldaña
Costs of HSCT in the United States have been widely reported, but complete information on costs in developing countries is lacking. We performed an analysis designed to assess the real, detailed costs of HSCT in Mexico. Using the database of the Current Accounts Department at our Institution, we performed a micro-costing based analysis of patients from 2010 through 2015 to obtain the overall cost of HSCT during the in-patient procedure and 2-month follow-up. One hundred five transplantations (57% autologous) were performed...
June 14, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28612279/prevalence-and-clinical-outcomes-of-hepatitis-b-virus-infection-in-patients-with-aplastic-anemia
#7
Pan Zhao, Qing Gao, Qiulian He, Jing Tan
The association of HBV infection with other hematopoietic diseases has been discussed previously. However, the clinical significance and clinical outcomes of HBV infection in AA patients have not been clarified. In this study, we sought to investigate the prevalence and related events of HBV in patients with AA who received immunosuppressive therapy. We retrospectively analyzed 245 patients with acquired AA. The HBsAg positivity rate was 14.69% in this group of AA patients. No significant difference was observed in the severity of AA patients with HBV infection and in those without (P = 0...
June 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28612278/efficacy-of-prophylactic-irradiation-to-the-contralateral-testis-for-patients-with-advanced-stage-primary-testicular-lymphoma-an-analysis-of-outcomes-at-a-single-institution
#8
Ryouji Tokiya, Eisaku Yoden, Kei Konishi, Nobuhiko Kamitani, Junichi Hiratsuka, Risa Koresawa, Tadashi Hirose, Fuminori Sano, Hirotoshi Tokunaga, Toshinori Kondo, Hideho Wada, Takashi Sugihara
BACKGROUND: Primary testicular lymphoma (PTL) is a rare, extranodal lymphoma that often relapses in the contralateral testis. We evaluated outcomes in patients with any stage of PTL who had received CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) with rituximab chemotherapy and prophylactic radiotherapy to the contralateral testis. METHODS: We retrospectively identified 15 patients (median age 66 years; range 39-81) diagnosed with diffuse large B-cell PTL in the period 2000-2014...
June 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28608231/extradural-plasmacytoma-with-central-nervous-system-involvement-in-newly-diagnosed-multiple-myeloma
#9
LETTER
Satoshi Kaito, Hideharu Muto, Shunta Takebayashi, Yutaka Otsubo, Shuichi Miyawaki, Kazuteru Ohashi
No abstract text is available yet for this article.
June 12, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28600720/comparisons-of-argatroban-to-lepirudin-and-bivalirudin-in-the-treatment-of-heparin-induced-thrombocytopenia-a-systematic-review-and-meta-analysis
#10
REVIEW
Zhengwu Sun, Xiaoyan Lan, Shen Li, Hongling Zhao, Zeyao Tang, Yalin Xi
To prevent thromboembolic events associated with heparin-induced thrombocytopenia (HIT), patients usually are treated with argatroban, lepirudin, and bivalirudin. Here, we conducted a meta-analysis of studies to comparing the treatment of HIT with the following direct thrombin inhibitor: argatroban versus lepirudin and argatroban versus bivalirudin. We systematically searched PubMed, Embase, and Cochrane Library database for relevant studies. The clinical outcomes were thromboembolic complication and bleeding...
June 9, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28597329/safety-and-efficacy-of-mogamulizumab-in-patients-with-adult-t-cell-leukemia-lymphoma-in-japan-interim-results-of-postmarketing-all-case-surveillance
#11
Kenji Ishitsuka, Satoshi Yurimoto, Kouichi Kawamura, Yukie Tsuji, Manabu Iwabuchi, Takeshi Takahashi, Kensei Tobinai
We present the interim results of a postmarketing all-case surveillance study in patients with C-C chemokine receptor 4 (CCR4)-positive, relapsed or refractory adult T-cell leukemia-lymphoma (ATL) treated with the anti-CCR4 monoclonal antibody mogamulizumab since its 2012 launch in Japan. The safety and efficacy analysis populations comprised 484 and 442 patients, respectively. The ATL subtype was acute in 58.9% and lymphoma in 34.2% of patients. All patients were scheduled to receive intravenous infusions of mogamulizumab (1...
June 9, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28597369/first-report-of-real-time-monitoring-of-coagulation-function-potential-and-igg-subtype-of-anti-fviii-autoantibodies-in-a-child-with-acquired-hemophilia-a-associated-with-streptococcal-infection-and-amoxicillin
#12
Masahiro Takeyama, Keiji Nogami, Takahiro Kajimoto, Kenichi Ogiwara, Tomoko Matsumoto, Midori Shima
We describe an 8-year-old boy with acquired hemophilia A (AHA) associated with streptococcal infection and amoxicillin. Laboratory data revealed low factor VIII activity (FVIII:C, 1.5 IU/dl), and FVIII inhibitor (15.9 BU/ml). Comprehensive coagulation function assays, including rotation thromboelastometry (ROTEM(®)), revealed a markedly prolonged clotting time. Thrombin and plasmin generation (TG/PG) appeared to be moderately impaired. The inhibitor epitope of his anti-FVIII autoantibody recognized light and heavy chains...
June 8, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28584963/steroid-resistant-autoimmune-myelofibrosis-in-a-patient-with-autoimmune-hepatitis-and-evans-syndrome-complicated-with-increased-expression-of-tgf-%C3%AE-in-the-bone-marrow-a-case-report
#13
Hiroshi Ohkawara, Miki Furukawa, Kazuhiko Ikeda, Akiko Shichishima-Nakamura, Masahiko Fukatsu, Takahiro Sano, Koki Ueda, Satoshi Kimura, Risa Kanai, Yuka Oka, Fumi Murakami, Osamu Suzuki, Yuko Hashimoto, Kazuei Ogawa, Takayuki Ikezoe
We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20(+)), T lymphocytes (CD3(+), CD8(+)), and plasma cells (CD138(+)). Immunohistochemical analysis revealed increased expression of transforming growth factor-β (TGF-β) in infiltrating lymphocytes and macrophages in the bone marrow. She was initially treated with oral prednisolone (PSL) for 2 months, which had a limited effect...
June 5, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28577208/erratum-to-primary-fungal-prophylaxis-in-acute-leukemia-patients-with-different-risk-factors-retrospective-analysis-from-the-caesar-study
#14
Rongli Zhang, Jing Chen, He Huang, Jun Ma, Fanyi Meng, Yongmin Tang, Jianda Hu, Xi Zhang, Yu Ji, Huisheng Ai, Yingmin Liang, Depei Wu, Xiaojun Huang, Mingzhe Han
No abstract text is available yet for this article.
June 2, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28555415/plasma-cell-myeloma-with-histiocyte-like-morphology
#15
LETTER
Jianfeng Zhu, Baishen Pan, Jiamei Yao, Wei Guo
No abstract text is available yet for this article.
May 29, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28555414/germline-etv6-mutations-and-predisposition-to-hematological-malignancies
#16
REVIEW
Simone Feurstein, Lucy A Godley
Patients with thrombocytopenia 5 have an autosomal dominant disorder of decreased platelet number with tendency to bleed, usually presenting in childhood, and have been found to have germline mutations in ETV6, which encodes a master hematopoietic transcription factor. Some patients who present similarly have inherited mutations in RUNX1 or ANKRD26. All three germline syndromes are also associated with a predisposition to myelodysplastic syndrome (MDS) and acute leukemia (AL). Since the first description of germline ETV6 mutations, 18 families have been reported...
May 29, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28550352/efficacy-and-safety-of-full-length-pegylated-recombinant-factor-viii-with-extended-half-life-in-previously-treated-patients-with-hemophilia-a-comparison-of-data-between-the-general-and-japanese-study-populations
#17
Keiji Nogami, Midori Shima, Katsuyuki Fukutake, Teruhisa Fujii, Masashi Taki, Tadashi Matsushita, Satoshi Higasa, Tetsuji Sato, Michio Sakai, Morio Arai, Haruhiko Uchikawa, Werner Engl, Brigitt Abbuehl, Barbara A Konkle
Rurioctocog alfa pegol (BAX 855) is a novel third-generation recombinant factor VIII whose active ingredient is chemically modified with polyethylene glycol. A global multicenter phase 2/3 study of the product in 137 patients (including 11 patients from Japan) with severe hemophilia A aged 12-65 years, reported an extended half-life and a good tolerability profile, as well as a significantly lower annualized bleeding rate in the prophylactic treatment arm than in the on-demand treatment arm. Using descriptive statistics, a post hoc analysis was performed to compare the pharmacokinetics, safety, and efficacy profiles of the product in the Japanese subpopulation and the overall population...
May 26, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28550350/recent-advances-in-the-study-of-immunodeficiency-and-dna-damage-response
#18
REVIEW
Tomohiro Morio
DNA breaks can be induced by exogenous stimuli or by endogenous stress, but are also generated during recombination of V, D, and J genes (V(D)J recombination), immunoglobulin class switch recombination (CSR). Among various DNA breaks generated, DNA double strand break (DSB) is the most deleterious one. DNA damage response (DDR) is initiated when DSBs are detected, leading to DNA break repair by non-homologous end joining (NHEJ). The process is critically important for the generation of diversity for foreign antigens; and failure to exert DNA repair leads to immunodeficiency such as severe combined immunodeficiency and hyper-IgM syndrome...
May 26, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28547672/myeloid-neoplasms-with-germline-ddx41-mutation
#19
REVIEW
Jesse J C Cheah, Christopher N Hahn, Devendra K Hiwase, Hamish S Scott, Anna L Brown
Recently, DDX41 mutations have been identified both as germline and acquired somatic mutations in families with multiple cases of late-onset myelodysplastic syndrome (MDS) and/or acute myeloid leukemia. The majority of germline mutations are frameshift mutations suggesting loss of function with DDX41 acting as a tumor suppressor, and there is a common somatic missense mutation found in a majority of germline mutated tumors. Clinically, DDX41 mutations lead to development of high-risk MDS at an age similar to that observed in sporadic cohorts, presenting a unique challenge to hematologists in recognizing the familial context...
May 25, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28534116/myeloid-neoplasms-with-germ-line-runx1-mutation
#20
REVIEW
Yoshihiro Hayashi, Yuka Harada, Gang Huang, Hironori Harada
Familial platelet disorder with propensity to myeloid malignancies (FPD/AML) is an autosomal dominant disorder characterized by quantitative and/or qualitative platelet defects with a tendency to develop a variety of hematological malignancies. Heterozygous germ line mutations in the RUNX1 gene are responsible genetic events for FPD/AML. Notably, about half of individuals in the family with germ line mutations in RUNX1 develop overt hematological malignancies. The latency is also relatively long as an average age at diagnosis is more than 30 years...
May 22, 2017: International Journal of Hematology
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