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A characteristic flow cytometric pattern with broad forward scatter and narrowed side scatter helps diagnose immune thrombocytopenia (ITP).
Abstract Various disorders cause severe thrombocytopenia, which can lead to critical hemorrhage. Procedures that rapidly support the diagnosis and risk factors for serious bleeding were explored, with a focus on immune thrombocytopenia (ITP). Twenty-five patients with thrombocytopenia, including 13 with newly diagnosed ITP, 3 with chronic ITP, 6 with aplastic anemia (AA), and 3 with other thrombocytopenia (one acute myeloid leukemia, one acute lymphoblastic leukemia, and one hemophagocytic lymphohistiocytosis), were reviewed. In addition to platelet-related parameters obtained by an automated hematology analyzer, ...
Source: International Journal of Hematology - April 16, 2018 Category: Hematology Authors: Araki R, Nishimura R, Kuroda R, Fujiki T, Mase S, Noguchi K, Ikawa Y, Maeba H, Yachie A Tags: Int J Hematol Source Type: research

EPOCH regimen as salvage therapy for adult T-cell leukemia-lymphoma.
Abstract Adult T-cell leukemia-lymphoma (ATL) is an intractable hematopoietic malignancy with a very poor prognosis. Although improved responses have been achieved through intensive chemotherapy in newly diagnosed patients with aggressive ATL, most patients suffer from relapse or disease recurrence, and an effective salvage therapy, especially for candidates for allogeneic hematopoietic stem cell transplantation (allo-HSCT), is yet to be established. The efficacy of the EPOCH regimen has been reported for several lymphoid malignancies; however, its efficacy for ATL has not been sufficiently evaluated. Here, we rep...
Source: International Journal of Hematology - April 12, 2018 Category: Hematology Authors: Toriyama E, Imaizumi Y, Taniguchi H, Taguchi J, Nakashima J, Itonaga H, Sato S, Ando K, Sawayama Y, Hata T, Fukushima T, Miyazaki Y Tags: Int J Hematol Source Type: research

A case of primary familial congenital polycythemia with a novel EPOR mutation: possible spontaneous remission/alleviation by menstrual bleeding.
Abstract A 10-year-old girl with persistent erythrocytosis and ruddy complexion was diagnosed with primary familial congenital polycythemia (PFCP) involving a novel heterozygous mutation of c.1220C>A, p.Ser407X in exon 8 of the erythropoietin receptor gene (EPOR). This mutation causes truncation of EPOR, resulting in loss of the cytoplasmic region, which is necessary for negative regulation of erythropoietin signal transmission. Genetic analysis showed that the mutated EPOR was inherited from her mother. Her mother had polycythemia and had undergone venesection several times when she was young, but her polycyth...
Source: International Journal of Hematology - April 5, 2018 Category: Hematology Authors: Toriumi N, Kaneda M, Hatakeyama N, Manabe H, Okajima K, Sakurai Y, Yamamoto M, Sarashina T, Ikuta K, Azuma H Tags: Int J Hematol Source Type: research

Chinese guidelines for treatment of adult primary immune thrombocytopenia.
e X, Zhang GS, Zhang L, Zheng CC, Zhou H, Zhou M, Zhou RF, Zhou ZP, Zhu HL, Zhu TN, Hou M Abstract Primary immune thrombocytopenia (ITP) is a bleeding disorder commonly encountered in clinical practice. The International Working Group (IWG) on ITP has published several landmark papers on terminology, definitions, outcome criteria, bleeding assessment, diagnosis, and management of ITP. The Chinese consensus reports for diagnosis and management of adult ITP have been updated to the 4th edition. Based on current consensus positions and new emerging clinical evidence, the thrombosis and hemostasis group of the Chinese...
Source: International Journal of Hematology - April 4, 2018 Category: Hematology Authors: Liu XG, Bai XC, Chen FP, Cheng YF, Dai KS, Fang MY, Feng JM, Gong YP, Guo T, Guo XH, Han Y, Hong LJ, Hu Y, Hua BL, Huang RB, Li Y, Peng J, Shu MM, Sun J, Sun PY, Sun YQ, Wang CS, Wang SJ, Wang XM, Wu CM, Wu WM, Yan ZY, Yang FE, Yang LH, Yang RC, Yang TH, Tags: Int J Hematol Source Type: research

Effects of plasma glycosyltransferase on the ABO(H) blood group antigens of human von Willebrand factor.
Abstract Von Willebrand factor (VWF) is one of the plasma protein carrying ABO(H) blood group antigens, but the combining process of these antigens is not clear. In the present study, we examined whether plasma glycosyltransferase affects the blood group antigens on VWF. VWF expressing H-antigen (H-VWF) from blood group O and bovine serum albumin conjugated with H-antigen (H-BSA) were incubated with recombinant α1-3-N-acetylgalactosaminyltransferase (rA-transferase) and A-plasma with or without an additional UDP-GalNAc. Transformed antigens were detected by western blotting and ELISA, using an anti-A antibod...
Source: International Journal of Hematology - April 4, 2018 Category: Hematology Authors: Kano T, Kondo K, Hamako J, Matsushita F, Sakai K, Matsui T Tags: Int J Hematol Source Type: research

Presepsin as a diagnostic marker of bacterial infections in febrile neutropenic pediatric patients with hematological malignancies.
Abstract Febrile neutropenia (FN) is often observed in hematological malignancies (HEM). Presepsin is also known as soluble CD14 subtype; it is a glycoprotein fragment derived from monocytes and macrophages. We aimed to evaluate the significance of presepsin and other biomarkers for diagnosis of bacteremia in children with HEM. Sixty pediatric patients with different HEM (acute lymphoblastic leukemia 36, acute myeloid leukemia 12, non-Hodgkin lymphoma 10, and Hodgkin disease 2). Thirty age and sex-matched healthy children serving as control were enrolled in this study. Estimation of presepsin, procalcitonin (PCT),...
Source: International Journal of Hematology - April 3, 2018 Category: Hematology Authors: Baraka A, Zakaria M Tags: Int J Hematol Source Type: research

A discrepancy between prothrombin time and Normotest (Hepaplastintest) results is useful for diagnosis of acquired factor V inhibitors.
Abstract Acquired coagulation factor inhibitors are rare. Among them, coagulation factor V (FV) inhibitor is particularly uncommon and presents with variable clinical manifestations. Certain acquired FV inhibitor patients have only mild bleeding or, in select cases, no symptoms at all, leading to spontaneous recovery. Others have life-threatening bleeding that requires medical attention. Thus, a prompt decision regarding diagnosis and clinical intervention is crucial for such patients. In five acquired FV inhibitor cases treated in our facility, each patient had a malignancy as an underlying disease and all unexpe...
Source: International Journal of Hematology - April 2, 2018 Category: Hematology Authors: Kadohira Y, Yamada S, Hayashi T, Morishita E, Asakura H, Ichinose A Tags: Int J Hematol Source Type: research

Clinical characteristics and outcomes of diffuse large B-cell lymphoma in adolescents and young adults.
In conclusion, disease characteristics and outcomes of DLBCL in AYA were nearly the same as those in older adults. PMID: 29605873 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - March 31, 2018 Category: Hematology Authors: Suzuki Y, Yano T, Suehiro Y, Iwasaki H, Hidaka M, Otsuka M, Sunami K, Inoue N, Sawamura M, Ito T, Iida H, Nagai H Tags: Int J Hematol Source Type: research

The hematopoietic stem cell diet.
Abstract Hematopoietic stem cells (HSCs) are responsible for sustaining life-long blood formation or hematopoiesis and are also used clinically in a form of bone marrow transplantation, a curative cellular therapy for a range of hematological diseases. HSCs are maintained throughout adult life by a complex biological niche or microenvironment, which is thought to be composed of a range of cellular, molecular, and metabolic components. The metabolic components of the HSC niche have become of increasing interest over the past few years. It is now well-recognized that metabolic activity is intimately linked to H...
Source: International Journal of Hematology - March 31, 2018 Category: Hematology Authors: Wilkinson AC, Yamazaki S Tags: Int J Hematol Source Type: research

The significance and application of vascular niche in the development and maintenance of hematopoietic stem cells.
Abstract Deriving hematopoietic stem cells (HSCs) from human pluripotent stem cells is one of major goals in stem cell and hematological research. To induce HSCs from human pluripotent stem cells, many attempts to mimic embryonic development through stepwise exposure to morphogens. HSCs arise from dorsal aorta of embryos then migrate and settle in the bone marrow. Development and maintenance of HSCs are controlled by the microenvironmental cues around the blood vessels (called vascular niche) through morphogens and cytokines. Vascular niche serves as a common mechanism from embryo development to life-long maintena...
Source: International Journal of Hematology - March 30, 2018 Category: Hematology Authors: Sugimura R Tags: Int J Hematol Source Type: research

PAS positivity of erythroid precursor cells is associated with a poor prognosis in newly diagnosed myelodysplastic syndrome patients.
We examined the PAS positivity of the bone marrow erythroblasts of 144 patients newly diagnosed with MDS; 26 (18.1%) of them had PAS-positive erythroblasts, whereas 118 (81.9%) did not. The PAS-positive group showed significantly poorer karyotypes as defined in the revised International Prognostic Scoring System (IPSS-R) and higher scores in age-adjusted IPSS-R (IPSS-RA) than the PAS-negative group. Overall survival (OS) and leukemia-free survival (LFS) were also significantly shorter in the PAS-positive group than in the PAS-negative group. Similar results were obtained when only high- and very high risk groups were analy...
Source: International Journal of Hematology - March 29, 2018 Category: Hematology Authors: Masuda K, Shiga S, Kawabata H, Takaori-Kondo A, Ichiyama S, Kamikubo Y Tags: Int J Hematol Source Type: research

CBL mutation and MEFV single-nucleotide variant are important genetic predictors of tumor reduction in glucocorticoid-treated patients with chronic myelomonocytic leukemia.
In conclusion, GC therapy effectively treats CMML cells harboring the MEFV SNV and CBL mutation by reducing inflammatory cytokine production and terminating prolonged STAT5 phosphorylation in the GM-CSF signaling pathway. PMID: 29600428 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - March 29, 2018 Category: Hematology Authors: Watanabe J, Sato K, Osawa Y, Horiuchi T, Kato S, Hikota-Saga R, Maekawa T, Yamamura T, Kobayashi A, Kobayashi S, Kimura F Tags: Int J Hematol Source Type: research

Bortezomib maintenance therapy in transplant-ineligible myeloma patients who plateaued after bortezomib-based induction therapy: a multicenter phase II clinical trial.
In conclusion, although maintenance therapy with biweekly bortezomib for up to 1 year was feasible, plateau-oriented bortezomib induction therapy followed by bortezomib maintenance therapy was not adequate in newly diagnosed transplant-ineligible MM patients. PMID: 29594921 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - March 28, 2018 Category: Hematology Authors: Isoda A, Murayama K, Ito S, Kohara Y, Iino M, Miyazawa Y, Matsumoto M, Handa H, Imai Y, Ishiguro T, Izumita W, Kitano K, Hirabayashi Y, Nakazawa H, Ishida F, Mitsumori T, Kirito K, Chou T, Murakami H, Kanshinetsu Multiple Myeloma Study Group Tags: Int J Hematol Source Type: research

Intensification of induction therapy and prolongation of maintenance therapy did not improve the outcome of pediatric Langerhans cell histiocytosis with single-system multifocal bone lesions: results of the Japan Langerhans Cell Histiocytosis Study Group-02 Protocol Study.
Abstract Langerhans cell histiocytosis (LCH) with single-system (SS) multifocal bone (MFB) lesions is rarely fatal, but patients may experience relapses and develop LCH-associated sequelae. To evaluate effect on outcomes of pediatric multifocal LCH, we tested a treatment protocol modified from the Japan Langerhans Cell Histiocytosis Study Group (JLSG)-96 study. We assessed the outcomes of all consecutive newly diagnosed pediatric patients with LCH with SS-MFB lesions who were treated with JLSG-02 protocol in 2002-2009. JLSG-02 was modified from JLSG-96 as follows: increased prednisolone dosage at the induction pha...
Source: International Journal of Hematology - March 28, 2018 Category: Hematology Authors: Morimoto A, Shioda Y, Imamura T, Kudo K, Kitoh T, Kawaguchi H, Goto H, Kosaka Y, Tsunematsu Y, Imashuku S, Japan LCH Study Group Tags: Int J Hematol Source Type: research

Perioperative safety and hemostatic efficacy of Advate ® in patients with hemophilia A in a postmarketing surveillance in Japan.
Perioperative safety and hemostatic efficacy of Advate® in patients with hemophilia A in a postmarketing surveillance in Japan. Int J Hematol. 2018 Mar 28;: Authors: Nogami K, Takedani H, Shima M, Yoshioka A, Matsushita T, Takamatsu J, Taki M, Fukutake K, Uchikawa H, Takagi H, Arai M, Engl W, Shirahata A Abstract Rurioctocog alfa (recombinant factor VIII: Advate®) is available for the control of bleeding among patients with hemophilia A in Japan. To evaluate the perioperative safety and hemostatic efficacy of Advate®, a postmarketing surveillance was conducted in Japanese patients undergoi...
Source: International Journal of Hematology - March 28, 2018 Category: Hematology Authors: Nogami K, Takedani H, Shima M, Yoshioka A, Matsushita T, Takamatsu J, Taki M, Fukutake K, Uchikawa H, Takagi H, Arai M, Engl W, Shirahata A Tags: Int J Hematol Source Type: research

Response to cyclosporine A and corticosteroids in adult patients with acquired pure red cell aplasia: serial experience at a single center.
Abstract To assess response to cyclosporine A, and/or corticosteroids, and possible factors influencing the response in adult patients with acquired pure red cell aplasia (PRCA). Clinical data from 42 cases were retrospectively analyzed. These patients received cyclosporine A (CsA), and/or corticosteroids (CS), or other immunosuppressive agents on becoming refractory and relapse. Thirty-nine patients were evaluated. Remission induction therapy included CsA (n = 16), CS (n = 13), CsA in combination with CS (n = 6), or other immunosuppressive agents (n = 4). In...
Source: International Journal of Hematology - March 27, 2018 Category: Hematology Authors: Wu X, Wang S, Lu X, Shen W, Qiao C, Wu Y, Lu R, Wang S, Zhang J, Hong M, Zhu Y, Li J, He G Tags: Int J Hematol Source Type: research

Treatment outcome of children with acute lymphoblastic leukemia: the Tokyo Children's Cancer Study Group (TCCSG) Study L04-16.
This report outlines the background characteristics and treatment outcomes of 1033 children with ALL treated according to a TCCSG platform. The 5-year event-free and overall survival (OS) rates for all children were 78.1 ± 1.3 and 89.6 ± 1.0%, respectively. The OS rate was significantly higher in children with B-cell precursor (BCP)-ALL (91.9 ± 1.0%, n = 916) than in those with T-ALL (71.9 ± 4.3%, n = 117, p 
Source: International Journal of Hematology - March 27, 2018 Category: Hematology Authors: Takahashi H, Kajiwara R, Kato M, Hasegawa D, Tomizawa D, Noguchi Y, Koike K, Toyama D, Yabe H, Kajiwara M, Fujimura J, Sotomatsu M, Ota S, Maeda M, Goto H, Kato Y, Mori T, Inukai T, Shimada H, Fukushima K, Ogawa C, Makimoto A, Fukushima T, Ohki K, Koh K, Tags: Int J Hematol Source Type: research

Influence of post-transplant mucosal-associated invariant T cell recovery on the development of acute graft-versus-host disease in allogeneic bone marrow transplantation.
We examined the impact of post-hematopoietic stem cell transplantation (HSCT) MAIT and iNKT cell recovery on the clinical outcomes of 69 patients who underwent allogeneic HSCT at Kyoto University Hospital. Multivariate analyses identified the absolute number of MAIT cells (
Source: International Journal of Hematology - March 26, 2018 Category: Hematology Authors: Kawaguchi K, Umeda K, Hiejima E, Iwai A, Mikami M, Nodomi S, Saida S, Kato I, Hiramatsu H, Yasumi T, Nishikomori R, Kondo T, Takaori-Kondo A, Heike T, Adachi S Tags: Int J Hematol Source Type: research

Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation.
Abstract A case of acquired hemophilia A (AHA) that developed in a patient with autoimmune pancreatitis (AIP) is presented. A 64-year-old woman was diagnosed with AIP in 2007. The symptoms resolved with prednisolone (PSL). Although the dose of PSL was tapered to 7.5 mg/day for maintenance, serum IgG4 levels remained high. She suddenly presented with subcutaneous bleeding in 2015. Her activated partial thromboplastin time was prolonged (80.0 s). A mixing test showed an inhibitor pattern, factor VIII (FVIII) activity was less than 1%, and FVIII inhibitor was 290 BU/mL. She was diagnosed with AHA. Her ...
Source: International Journal of Hematology - March 26, 2018 Category: Hematology Authors: Narazaki T, Haji S, Nakashima Y, Tsukamoto Y, Tsuda M, Takamatsu A, Ohno H, Matsushima T, Matsumoto T, Nogami K, Shima M, Shiratsuchi M, Ogawa Y Tags: Int J Hematol Source Type: research

Nationwide survey of therapy-related leukemia in childhood in Japan.
We report a retrospective nationwide survey of patients diagnosed between 2000 and 2013 in Japan. We identified 43 patients with pediatric t-leukemia; 33 had t-acute myeloid leukemia (t-AML), eight had t-acute lymphoblastic leukemia (t-ALL) and two had t-acute undifferentiated leukemia. Median age at onset and latency were 12 years and 3.8 years, respectively, consistent with previous reports. Of t-AML patients, 63.6% harbored topoisomerase II inhibitor (topo II)-related genetic abnormalities, while only 12.5% of t-ALL patients had such alterations, suggesting that topo II is not key to t-ALL leukemogenesis. The ...
Source: International Journal of Hematology - March 24, 2018 Category: Hematology Authors: Imamura T, Taga T, Takagi M, Kawasaki H, Koh K, Taki T, Adachi S, Manabe A, Ishida Y, Leukemia/Lymphoma Committee, Japanese Society of Pediatric Hematology Oncology (JSPHO) Tags: Int J Hematol Source Type: research

CD3+ CD56+ EBER1+ atypical extraosseous plasmacytoma of the nasal cavity.
We present a case of atypical extraosseous plasmacytoma (EP) with a plasmablastic morphology and CD3+ CD56+ Epstein-Barr virus-encoded RNA-1 (EBER1)+ phenotypes of the nasal cavity. A 51-year-old male attended a local hospital with a tumor located in his left nasal cavity that was histologically diagnosed as extranodal NK/T cell lymphoma, nasal type. We reviewed the specimens and re-biopsied the tumor, and confirmed that the tumor was positive for CD3, CD56, and EBER1; however, the tumor showed a plasmablastic morphology without necrosis and angiocentricity, and was positive for CD138, but negative for CD20 and PAX5. Given...
Source: International Journal of Hematology - March 23, 2018 Category: Hematology Authors: Ando Y, Maeshima AM, Fukuhara S, Makita S, Munakata W, Suzuki T, Maruyama D, Taniguchi H, Izutsu K Tags: Int J Hematol Source Type: research

Early lymphocyte recovery predicts clinical outcome after HSCT with mycophenolate mofetil prophylaxis in the Japanese population.
Abstract Immune reconstitution affects clinical outcomes after allogeneic hematopoietic stem cell transplantation (HSCT), and it has been suggested that lymphocyte recovery affects survival after HSCT. However, few studies have examined lymphocyte recovery in Asian patients who received mycophenolate mofetil (MMF) prophylaxis for graft-versus-host disease. We retrospectively evaluated early lymphocyte recovery after HSCT among Japanese adults who received MMF prophylaxis. Patients were divided into two groups according to their median absolute lymphocyte count (ALC) on day 28 after HSCT as follows: the "low A...
Source: International Journal of Hematology - March 22, 2018 Category: Hematology Authors: Kurata K, Yakushijin K, Mizuno I, Gomyo H, Okamura A, Ichikawa H, Sakai R, Mizutani Y, Kakiuchi S, Miyata Y, Kitao A, Sanada Y, Inui Y, Uryu K, Kawamoto S, Sugimoto T, Yamamoto K, Ito M, Matsuoka H, Murayama T, Minami H Tags: Int J Hematol Source Type: research

Impact of the D-index deduced from duration and intensity of neutropenia following chemotherapy on the risk of invasive fungal infection in pediatric acute myeloid leukemia.
Abstract Pediatric patients with acute myeloid leukemia (AML) are at high risk of invasive fungal infection (IFI). In adult patients, the D-index, which reflects the duration and intensity of neutropenia, was reported as a predictive factor of IFI after induction therapy for AML. The aim of this study was to assess whether the D-index is a predictive factor for IFI in pediatric AML. We define the D-index as the area over the neutrophil curve during neutropenia. Ninety-two courses of chemotherapy performed for 24 consecutive patients with AML undergoing chemotherapy at Sapporo Hokuyu Hospital between April 2007 and...
Source: International Journal of Hematology - March 21, 2018 Category: Hematology Authors: Sano H, Kobayashi R, Suzuki D, Hori D, Kishimoto K, Kobayashi K Tags: Int J Hematol Source Type: research

The role of telomere binding molecules for normal and abnormal hematopoiesis.
Abstract In order to maintain the homeostasis of the hematopoietic system, hematopoietic stem cells (HSCs) need to be maintained while slowly dividing over their lifetime. However, repeated cell divisions lead to the gradual accumulation of DNA damage and ultimately impair HSC function. Since telomeres are particularly fragile when subjected to replication stress, cells have several defense machinery to protect telomeres. Moreover, HSCs must protect their genome against possible DNA damage, while maintaining telomere length. A group of proteins called the shelterin complex are deeply involved in this two-way role,...
Source: International Journal of Hematology - March 17, 2018 Category: Hematology Authors: Hosokawa K, Arai F Tags: Int J Hematol Source Type: research

Clofarabine followed by haploidentical stem cell transplant using fludarabine, busulfan, and total-body irradiation with post-transplant cyclophosphamide in non-remission AML.
Abstract Approximately 30-40% of patients with acute myeloid leukemia (AML) experience induction failures. In these patients who do not achieve remission with two cycles of standard induction therapies, the probability of achieving remission with subsequent inductions is very limited. Hematopoietic stem cell transplantation (HSCT) is the only curative option for these patients, but high relapse rate and transplant-related mortality often preclude them to proceed to transplant. Thus, AML not in remission at time of HSCT remains a huge unmet need in current HSCT practice, particularly if the patient does not have an...
Source: International Journal of Hematology - March 14, 2018 Category: Hematology Authors: Rakszawski K, Miki K, Claxton D, Wagner H, Shike H, Mineishi S, Naik S Tags: Int J Hematol Source Type: research

EBV-positive PEL-like lymphoma that developed in the course of antisynthetase syndrome treated with tacrolimus.
We describe a case of PEL-like lymphoma that developed in the course of antisynthetase syndrome and was treated with tacrolimus. A single dose of systemic chemotherapy did not improve lymphomatous effusion, whereas the discontinuation of tacrolimus resulted in the long-term remission of this disease. PMID: 29516290 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - March 7, 2018 Category: Hematology Authors: Okada K, Asakura S, Yano T, Kishimoto T Tags: Int J Hematol Source Type: research

T-cell-replete haploidentical stem cell transplantation using low-dose antithymocyte globulin in children with relapsed or refractory acute leukemia.
Abstract We evaluated the efficacy and toxicity of T-cell-replete haploidentical stem cell transplantation (TCR-haploSCT) using low-dose antithymocyte globulin (ATG) in children with refractory/relapsed (R/R) acute leukemia. From October 2009 to April 2016, 39 consecutive patients with R/R acute leukemia who underwent TCR-haploSCT were included. At the time of TCR-haploSCT, 17 patients were in complete remission (CR), but 22 had active disease. Thirty-three patients received a myeloablative regimen and six received a reduced-intensity conditioning regimen. Graft-versus-host disease (GvHD) prophylaxis comprised tac...
Source: International Journal of Hematology - March 5, 2018 Category: Hematology Authors: Sano H, Mochizuki K, Kobayashi S, Ohara Y, Ito M, Waragai T, Takahashi N, Ikeda K, Ohto H, Kikuta A Tags: Int J Hematol Source Type: research

Epidemiology of malignant lymphoma and recent progress in research on adult T-cell leukemia/lymphoma in Japan.
Abstract The morbidity and mortality of disease vary according to the region and may also change over time. The morbidity and mortality of malignant lymphoma are also affected by differences in ethnicity, lifestyle habits, geographical area, and time period. Increasing research on malignant lymphoma has focused on its pathophysiology, diagnosis, and therapeutic treatment. Recent improvements in the accuracy of clinical study, technologies such as next-generation sequencing, and the development of targeted molecular agents have also resulted in a number of excellent studies. This review summarizes the epidemiology ...
Source: International Journal of Hematology - March 3, 2018 Category: Hematology Authors: Miyoshi H, Ohshima K Tags: Int J Hematol Source Type: research

Current status and progress of lymphoma research in East Asian countries: Introduction and planning.
PMID: 29492832 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 28, 2018 Category: Hematology Authors: Suzumiya J Tags: Int J Hematol Source Type: research

Thrombohemorrhagic events, disease progression, and survival in polycythemia vera and essential thrombocythemia: a retrospective survey in Miyazaki prefecture, Japan.
Abstract Polycythemia vera (PV) and essential thrombocythemia (ET) are associated with life-threatening thrombohemorrhagic events, and disease progression and development of non-hematological malignancies also reduce long-term survival. We retrospectively surveyed thrombohemorrhagic events and overall survival (OS) in 62 PV and 117 ET patients. The cumulative incidences of thrombohemorrhagic events in PV and ET patients were 11.3 and 10.3%, and the incidence rates were 2.42 and 1.85 per 100 person-years. The combined incidence rates of disease progression and development of non-hematological maligna...
Source: International Journal of Hematology - February 27, 2018 Category: Hematology Authors: Kamiunten A, Shide K, Kameda T, Sekine M, Kubuki Y, Ito M, Toyama T, Kawano N, Marutsuka K, Maeda K, Takeuchi M, Kawano H, Sato S, Ishizaki J, Akizuki K, Tahira Y, Shimoda H, Hidaka T, Yamashita K, Matsuoka H, Shimoda K Tags: Int J Hematol Source Type: research

miR-150 regulates B lymphocyte in autoimmune hemolytic anemia/Evans syndrome by c-Myb.
The objective of the study was to study the regulation of B lymphocytes in patients with autoimmune hemolytic anemia (AIHA)/Evans syndrome. From October 2015 to May 2016, 35 patients with AIHA/Evans in the Department of Hematology, Tianjin Medical University General Hospital were enrolled into this study. c-Myb mRNA and miR-150 expression in B lymphocytes were measured using real-time PCR (RT-PCR). Correlation between c-Myb and miR-150 and clinical parameters in patients with AIHA/Evans were analyzed. c-Myb mRNA expression in hemolysis patients was significantly higher than in remission patients and CLL patients, negativel...
Source: International Journal of Hematology - February 27, 2018 Category: Hematology Authors: Xing L, Xu W, Qu Y, Zhao M, Zhu H, Liu H, Wang H, Su X, Shao Z Tags: Int J Hematol Source Type: research

Atypical erythroblastosis in a patient with Diamond-Blackfan anemia who developed del(20q) myelodysplasia.
Abstract Diamond-Blackfan anemia (DBA) is a congenital red cell aplasia arising from ribosomal protein (RP) defects. Affected patients present with neonatal anemia, occasional dysmorphism, and cancer predisposition. An anemic newborn was diagnosed with DBA due to RPL5 mutation (c.473_474del, p.K158SfsX26). Refractory anemia required regular transfusions and iron chelation therapy. Pancytopenia occurred at age 16 years. Bone-marrow studies showed myelodysplasia, erythroblastosis, and clonal evolution of del(20)(q11.2q13.3). Severe anemia required transfusions. Del(20q), including the L3MBTL1 gene, is reported ...
Source: International Journal of Hematology - February 23, 2018 Category: Hematology Authors: Sonoda M, Ishimura M, Ichimiya Y, Terashi E, Eguchi K, Sakai Y, Takada H, Hama A, Kanno H, Toki T, Ito E, Ohga S Tags: Int J Hematol Source Type: research

Lumbosacral polyradiculopathy after intrathecal chemotherapy in pediatric acute lymphoblastic leukemia.
PMID: 29470812 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 22, 2018 Category: Hematology Authors: Suematsu M, Imamura T, Chiyonobu T, Osone S, Hosoi H Tags: Int J Hematol Source Type: research

Mutational subtypes of JAK2 and CALR correlate with different clinical features in Japanese patients with myeloproliferative neoplasms.
In conclusion, Japanese patients with MPNs grouped by different mutation subtypes exhibit characteristics similar to those of their Western counterparts. In addition, ET and pre-PMF patients show different characteristics, even when restricted to JAK2V617F-mutated patients. PMID: 29464483 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 20, 2018 Category: Hematology Authors: Misawa K, Yasuda H, Araki M, Ochiai T, Morishita S, Shirane S, Edahiro Y, Gotoh A, Ohsaka A, Komatsu N Tags: Int J Hematol Source Type: research

Characteristics and outcome of chronic myeloid leukemia patients with E255K/V BCR-ABL kinase domain mutations.
Abstract Kinase domain (KD) mutations of ABL1 represent the most common resistance mechanism to tyrosine kinase inhibitors (TKI) in CML. Besides T315I, mutations in codon 255 are highly resistant mutations in vitro to all TKI. We aimed to study the incidence, prognosis, and response to treatment in patients with E255K/V. We evaluated 976 patients by sequencing of BCR-ABL1 fusion transcript for ABL1 KD mutations. We identified KD mutations in 381 (39%) patients, including E255K/V in 48 (13% of all mutations). At mutation detection, 14 patients (29%) were in chronic phase (CP), 12 (25%) in accelerated phase (AP), an...
Source: International Journal of Hematology - February 20, 2018 Category: Hematology Authors: Naqvi K, Cortes JE, Luthra R, O'Brien S, Wierda W, Borthakur G, Kadia T, Garcia-Manero G, Ravandi F, Rios MB, Dellasala S, Pierce S, Jabbour E, Patel K, Kantarjian H Tags: Int J Hematol Source Type: research

Guest editorial: advances in immunotherapy for hematological malignancies.
PMID: 29427173 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 9, 2018 Category: Hematology Authors: Kadowaki N Tags: Int J Hematol Source Type: research

Re: Dabigatran overdose: a case report of acute hepatitis. Extracorporeal treatment.
PMID: 29423560 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 8, 2018 Category: Hematology Authors: Caruso K, Kanter M Tags: Int J Hematol Source Type: research

Clonal dynamics in a case of acute monoblastic leukemia that later developed myeloproliferative neoplasm.
We report here an acute monoblastic leukemia (AMoL) patient who later developed an unclassifiable myeloproliferative neoplasm (MPN-U). Whole-exome sequencing and cluster analysis demonstrated the presence of three distinct major clones during the clinical course: (1) an AMoL clone with ASXL1, CBL, and NPM1 somatic mutations, likely associated with the pathogenesis, and GATA2, SRSF2, and TET2 mutations, (2) an AMoL remission clone, with mutated GATA2, SRSF2, and TET2 only (possibly the founding clone (pre-leukemic HSC) that survived chemotherapy), (3) a small subclone which had JAK2 mutation during the AMoL remission, appea...
Source: International Journal of Hematology - February 7, 2018 Category: Hematology Authors: Sato S, Itonaga H, Taguchi M, Sawayama Y, Imanishi D, Tsushima H, Hata T, Moriuchi Y, Mishima H, Kinoshita A, Yoshiura KI, Miyazaki Y Tags: Int J Hematol Source Type: research

Vwf K1362A resulted in failure of protein synthesis in mice.
Abstract Von Willebrand factor (VWF) is synthesized in megakaryocytes and endothelial cells (ECs) and has two main roles: to carry and protect coagulation factor VIII (FVIII) from degradation by forming VWF-FVIII complex; and to mediate platelet adhesion and aggregation at sites of vascular injury. Previous research using the HEK293 cell line revealed that the VWF K1362 mutation interacted directly with platelet glycoprotein Ib (GPIb). Vwf K1362A knock-in (KI) mice were therefore generated to verify the in vivo function of residue 1362 in binding to platelet GPIb. The Cre-loxP system was employed to introduce the ...
Source: International Journal of Hematology - February 1, 2018 Category: Hematology Authors: Sanda N, Suzuki N, Suzuki A, Kanematsu T, Kishimoto M, Hasuwa H, Takagi A, Kojima T, Matsushita T, Nakamura S Tags: Int J Hematol Source Type: research

Improved progression-free and event-free survival in myeloma patients undergoing PBSCH receiving a cyclophosphamide  + G-CSF regimen than G-CSF alone.
The objective of the present study was to evaluate the anti-myeloma effect of the PBSCH regimen including HD-CY. We retrospectively assessed harvesting efficiency, complications, and anti-myeloma effects in 115 patients receiving HD-CY + G-CSF (HD-CY group) and 32 patients receiving G-CSF alone (G-alone group). We collected > 2 × 106 CD34-positive cells/kg from 93 and 75% of patients in the HD-CY and G-alone groups, respectively (P = 0.0079). The mean HSC count was also higher in the HD-CY group. No severe complications were observed in the G-alone group, whereas 66% of pati...
Source: International Journal of Hematology - January 31, 2018 Category: Hematology Authors: Tanimura A, Hirai R, Nakamura M, Takeshita M, Hagiwara S, Miwa A Tags: Int J Hematol Source Type: research

Cloning and expansion of antigen-specific T cells using iPS cell technology: development of "off-the-shelf" T cells for the use in allogeneic transfusion settings.
Cloning and expansion of antigen-specific T cells using iPS cell technology: development of "off-the-shelf" T cells for the use in allogeneic transfusion settings. Int J Hematol. 2018 Jan 31;: Authors: Kawamoto H, Masuda K, Nagano S, Maeda T Abstract Recent advances in adoptive immunotherapy using cytotoxic T lymphocytes (CTLs) have led to moderate therapeutic anti-cancer effects in clinical trials. However, a critical issue, namely that CTLs collected from patients are easily exhausted during expansion culture, has yet to be solved. To address this issue, we have been developing a strategy ...
Source: International Journal of Hematology - January 31, 2018 Category: Hematology Authors: Kawamoto H, Masuda K, Nagano S, Maeda T Tags: Int J Hematol Source Type: research

Current status and progress of lymphoma management in China.
Abstract Lymphoma is a large group of lymphoid hematopoietic malignancies including Hodgkin lymphoma and non-Hodgkin's lymphoma. The various subtypes of lymphoma are different in clinical features, response to treatment and prognoses. The relative frequency of specific subtypes of lymphoma varies geographically. The mature T cell lymphoma is much more common in East Asia compared with Western countries. Chemotherapy plays an important role in the treatment of lymphoma. With advances in understanding the biology and genetics of lymphoma, many new agents are used in the treatment of lymphoma. In mainland China, some...
Source: International Journal of Hematology - January 31, 2018 Category: Hematology Authors: Shi Y Tags: Int J Hematol Source Type: research

Favorable survival in lung transplant recipients on preoperative low-dose, as compared to high-dose corticosteroids, after hematopoietic stem cell transplantation.
In this study, we assessed the effect of preoperative corticosteroid therapy on long-term survival in patients undergoing LT after HSCT. We retrospectively investigated data from 13 patients who had received high-dose corticosteroid therapy and nine who had received low-dose corticosteroid therapy prior to LT for pulmonary complications after HSCT. Other than the preoperative corticosteroid dose, patient clinical characteristics did not differ. The incidence of postoperative complications within the first year after LT was significantly lower in the low-dose corticosteroid group (p = 0.026). Survival after LT was...
Source: International Journal of Hematology - January 30, 2018 Category: Hematology Authors: Sugimoto S, Miyoshi K, Kurosaki T, Otani S, Yamane M, Kobayashi M, Oto T Tags: Int J Hematol Source Type: research

Effects of eculizumab treatment on quality of life in patients with paroxysmal nocturnal hemoglobinuria in Japan.
Abstract In paroxysmal nocturnal hemoglobinuria (PNH), various symptoms due to intravascular hemolysis exert a negative impact on patients' quality of life (QOL). To determine clinical factors related with improvements in QOL in PNH patients treated, we analyzed changes in QOL scales in PNH patients treated with eculizumab based on data collected from post-marketing surveillance in Japan. Summary statistics were obtained using figures from QOL scoring systems and laboratory values, and evaluated by t test. One-year administration of eculizumab improved the most QOL items in comparison with the baseline. In particu...
Source: International Journal of Hematology - January 30, 2018 Category: Hematology Authors: Ueda Y, Obara N, Yonemura Y, Noji H, Masuko M, Seki Y, Wada K, Matsuda T, Akiyama H, Ikezoe T, Chiba S, Kanda Y, Kawaguchi T, Shichishima T, Nakakuma H, Okamoto S, Nishimura JI, Kanakura Y, Ninomiya H Tags: Int J Hematol Source Type: research

Genetic analysis of a novel missense mutation (Gly542Ser) with factor XII deficiency in a Chinese patient of consanguineous marriage.
Abstract Coagulation factor XII deficiency is a rare autosomal recessive disorder, which could be found in a consanguineous family. We studied a Chinese family in which the activated partial thromboplastin time (APTT) of the proband had clearly prolonged up to 101.7 s, associated with low FXII activity of 3% and FXII antigen  A point mutation in exon 14, causing a novel Gly542Ser mutation in the catalytic domain. The results of the conservatism and bioinformatics analyses both indicated that the mutation likely affects the function of the protein. Additional expression studies in COS-7 cells showed that ...
Source: International Journal of Hematology - January 30, 2018 Category: Hematology Authors: Zou A, Wang M, Jin Y, Cheng X, Su K, Yang L Tags: Int J Hematol Source Type: research

Continuous infusions of B domain-truncated recombinant factor VIII, turoctocog alfa, for orthopedic surgery in severe hemophilia A: first case report.
We describe a 43-year-old patient with severe hemophilia A who required arthroscopic synovectomy of the right elbow and arthrodesis of the right ankle. He was treated with a bolus injection of turoctocog alfa (36 IU/kg) immediately before operation, followed by CI (infusion rate; 2.9 IU/kg/h) to maintain FVIII activity > 80 IU/dl throughout the perioperative period. Surgery was completed successfully with uncomplicated hemostatic control. CIs were continued until post-operative day (POD) 4. Further bolus injections were given from POD5. No anti-FVIII inhibitor has been detected post-operation. T...
Source: International Journal of Hematology - January 30, 2018 Category: Hematology Authors: Takeyama M, Nogami K, Kobayashi R, Ogiwara K, Taniguchi A, Nakanishi Y, Inagaki Y, Tanaka Y, Shima M Tags: Int J Hematol Source Type: research

β-Thalassemia intermedia: a comprehensive overview and novel approaches.
β-Thalassemia intermedia: a comprehensive overview and novel approaches. Int J Hematol. 2018 Jan 29;: Authors: Asadov C, Alimirzoeva Z, Mammadova T, Aliyeva G, Gafarova S, Mammadov J Abstract β-Thalassemia intermedia is a clinical condition of intermediate gravity between β-thalassemia minor, the asymptomatic carrier, and β-thalassemia major, the transfusion-dependent severe anemia. It is characterized by a significant clinical polymorphism, which is attributable to its genetic heterogeneity. Ineffective erythropoiesis, chronic anemia, and iron overload contribute to the clinical c...
Source: International Journal of Hematology - January 29, 2018 Category: Hematology Authors: Asadov C, Alimirzoeva Z, Mammadova T, Aliyeva G, Gafarova S, Mammadov J Tags: Int J Hematol Source Type: research

Pomalidomide with or without dexamethasone for relapsed/refractory multiple myeloma in Japan: a retrospective analysis by the Kansai Myeloma Forum.
In conclusion, POM and POM/DEX showed substantial efficacy against RRMM, but new combination therapies with POM are needed to improve efficacy further without causing hematologic toxicities. PMID: 29380179 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 29, 2018 Category: Hematology Authors: Matsumura-Kimoto Y, Kuroda J, Kaneko H, Kamitsuji Y, Fuchida SI, Nakaya A, Shibayama H, Uoshima N, Yokota I, Uchiyama H, Yagi H, Kosugi S, Matsui T, Ishikawa J, Matsuda M, Ohta K, Iida M, Tanaka H, Kobayashi M, Wada K, Shimazaki C, Nomura S, Imada K, Hino Tags: Int J Hematol Source Type: research

Classical Hodgkin lymphoma type post-transplant lymphoproliferative disorder in a kidney transplant recipient: a diagnostic pitfall.
We report a case of classical Hodgkin lymphoma type post-transplant lymphoproliferative disorder (HL-PTLD) after kidney transplantation to highlight the difficulty of distinguishing this disorder from Hodgkin lymphoma-like PTLD (HL-like PTLD). Through this case report and literature review, we seek to clarify definitive pathologic features to differentiate these two conditions. A 38-year-old male kidney transplant recipient who had been receiving immunosuppressants was admitted to our hospital with unidentified high fever. Computed tomography images and blood tests indicated a lymphoproliferative disorder. Abdominal lymph ...
Source: International Journal of Hematology - January 29, 2018 Category: Hematology Authors: Mohri T, Ikura Y, Hirakoso A, Okamoto M, Hishizawa M, Takaori-Kondo A, Kato S, Nakamura S, Yoshimura K, Okabe H, Iwai Y Tags: Int J Hematol Source Type: research

Novel findings from the Asian Lymphoma Study Group: focus on T and NK-cell lymphomas.
Abstract T and NK-cell lymphomas are aggressive neoplasms with a unique epidemiological distribution, demonstrating higher prevalence in Asian countries compared to the West. Through the efforts of international collaboration, significant progress has been made especially on the biological understanding and clinical management of rare lymphoma subtypes including NK/T-cell lymphomas and monomorphic epitheliotropic intestinal T-cell lymphoma. In this review, we summarize the current status of lymphoma research conducted by the Asian Lymphoma Study Group and highlight key updates on the advancement of T and...
Source: International Journal of Hematology - January 29, 2018 Category: Hematology Authors: Chan JY, Lim ST Tags: Int J Hematol Source Type: research