Log in to search using one of your social media accounts:

 

Protective effect of a newly developed fucose-deficient recombinant antithrombin against histone-induced endothelial damage.
In conclusion, both recombinant and plasma-derived antithrombin can protect vascular endothelial cells. Recombinant antithrombin may represent a useful new therapeutic agent for sepsis-associated vascular damage. PMID: 29353463 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 20, 2018 Category: Hematology Authors: Iba T, Hirota T, Sato K, Nagaoka I Tags: Int J Hematol Source Type: research

Risk-stratified therapy for children with FLT3-ITD-positive acute myeloid leukemia: results from the JPLSG AML-05 study.
chi S Abstract Acute myeloid leukemia harboring internal tandem duplication of FMS-like tyrosine kinase 3 (AMLFLT3-ITD) is associated with poor prognosis. We evaluated the results of the AML-05 study, in which all AMLFLT3-ITD patients were assigned to receive hematopoietic stem cell transplantation (HSCT) in the first remission (1CR). We also investigated the effects of additional genetic alterations on FLT3-ITD. The 5-year overall survival (OS) and event-free survival (EFS) rates among the 47 AMLFLT3-ITD patients were 42.2 and 36.8%, respectively. The 5-year disease-free survival rate among 29 patients without in...
Source: International Journal of Hematology - January 12, 2018 Category: Hematology Authors: Shimada A, Iijima-Yamashita Y, Tawa A, Tomizawa D, Yamada M, Norio S, Watanabe T, Taga T, Iwamoto S, Terui K, Moritake H, Kinoshita A, Takahashi H, Nakayama H, Koh K, Goto H, Kosaka Y, Saito AM, Kiyokawa N, Horibe K, Hara Y, Oki K, Hayashi Y, Tanaka S, Ad Tags: Int J Hematol Source Type: research

Circulating level of Th17 cells is associated with sensitivity to glucocorticoids in patients with immune thrombocytopenia.
Abstract Glucocorticoids are a widely recognized first-line therapy for immune thrombocytopenia (ITP). However, some patients are unresponsive to glucocorticoid therapy for reasons that remain unclear. Accumulating evidence suggests that CD4+ T-cell abnormalities play a crucial role in the development of ITP. In the present study, we investigated peripheral blood CD4+ T cells, Th17-associated cytokines, and the mRNA expression level of Th17 transcription factor-RORγt-in patients with newly-diagnosed ITP before glucocorticoid therapy. The study involved 27 newly-diagnosed patients. Th17-cell levels in the per...
Source: International Journal of Hematology - January 11, 2018 Category: Hematology Authors: Zhang Y, Ma T, Zhou X, Chen J, Li J Tags: Int J Hematol Source Type: research

Deferasirox for the treatment of iron overload after allogeneic hematopoietic cell transplantation: multicenter phase I study (KSGCT1302).
Abstract The aim of this study was to assess the safety and optimal dose of deferasirox for the treatment of iron overload after allogeneic hematopoietic cell transplantation (HCT). The primary endpoint was the maximum tolerated dose of deferasirox that was determined by the intrapatient dose escalation methods. A total of 16 patients with post-HCT iron overload were enrolled in the study. After excluding one case of early relapse, 15 remained evaluable. Their median age was 42 years (range 22-68). Median time from HCT to deferasirox administration was 9 months (range 6-84). Deferasirox was started at a ...
Source: International Journal of Hematology - January 5, 2018 Category: Hematology Authors: Tachibana T, Kanda J, Machida S, Saito T, Tanaka M, Najima Y, Koyama S, Miyazaki T, Yamamoto E, Takeuchi M, Morita S, Kanda Y, Kanamori H, Okamoto S, Kanto Study Group for Cell Therapy (KSGCT) Tags: Int J Hematol Source Type: research

Mild renal dysfunction defined by creatinine clearance rate has limited impact on clinical outcomes after allogeneic hematopoietic stem cell transplantation.
Abstract Creatinine clearance rate (Ccr) is a more accurate indicator of renal function than serum creatinine. Data are sparse regarding the prognostic value of renal impairment calculated using Ccr in patients who undergo allogeneic hematopoietic stem cell transplantation (allo-HSCT). We performed a retrospective analysis of 185 patients who underwent allo-HSCT. These patients were divided into two groups by Ccr (ml/min) before transplantation; one showed normal renal function (Ccr ≥ 60, n = 156) and the other showed mild renal dysfunction (30 ≤ Ccr 
Source: International Journal of Hematology - January 4, 2018 Category: Hematology Authors: Ikegawa S, Matsuoka KI, Inomata T, Ikeda N, Sugiura H, Kuroi T, Asano T, Yoshida S, Nishimori H, Fujii N, Kondo E, Maeda Y, Tanimoto M Tags: Int J Hematol Source Type: research

Efficacy and safety of interferon alpha for essential thrombocythemia during pregnancy: two cases and a literature review.
We present the cases of two pregnant women with ET treated with interferon (IFN)-alpha. Each case showed a marked platelet decrease, from values within normal limits at the time of delivery, with no severe adverse events. To clarify the efficacy and safely of IFN alpha for ET during pregnancy, we performed a literature review. A total of 43 pregnant women with ET were ultimately identified from 12 articles and the present cases. IFN-alpha therapy decreased platelet counts to normal levels at birth in many cases, and there were no adverse events that required the discontinuation of IFN-alpha treatment. Overall, 93% of pregn...
Source: International Journal of Hematology - December 30, 2017 Category: Hematology Authors: Sakai K, Ueda A, Hasegawa M, Ueda Y Tags: Int J Hematol Source Type: research

Rearrangement of VPS13B, a causative gene of Cohen syndrome, in a case of RUNX1-RUNX1T1 leukemia with t(8;12;21).
Abstract Variant chromosomal translocations associated with t(8;21) are observed in 3-4% of acute myeloid leukemia (AML) cases with a RUNX1-RUNX1T1 fusion gene. However, the molecular events that occur in variants of t(8;21) are not well characterized. In the present study, we report genetic features of a variant three-way translocation of t(8;12;21)(q22;p11;q22) in a patient with AML. In this patient, leukemia cells lacked azurophilic granules, which does not correspond with the classic features of t(8;21). RNA-seq analysis revealed that TM7SF3 at 12p11 was fused to VPS13B at 8q22 and VPS13B to RUNX1, in addition...
Source: International Journal of Hematology - December 20, 2017 Category: Hematology Authors: Abe A, Yamamoto Y, Katsumi A, Okamoto A, Tokuda M, Inaguma Y, Yamamoto K, Yanada M, Kanie T, Tomita A, Akatsuka Y, Okamoto M, Kameyama T, Mayeda A, Emi N Tags: Int J Hematol Source Type: research

New immunotherapy-based approach in allogeneic hematopoietic stem cell transplantation.
PMID: 29260506 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - December 19, 2017 Category: Hematology Authors: Maeda Y Tags: Int J Hematol Source Type: research

Safety and efficacy of daratumumab in combination with bortezomib and dexamethasone in Japanese patients with relapsed or refractory multiple myeloma.
Abstract Daratumumab in combination with bortezomib and dexamethasone (DVd) has demonstrated longer progression-free survival than combination of bortezomib and dexamethasone in patients with relapsed or refractory multiple myeloma (RRMM). In this multicenter, open-label, phase-1 study, the safety, efficacy, and pharmacokinetics (PK) of DVd were evaluated in Japanese patients with RRMM. Eight patients with RRMM aged between 54 and 82 years were enrolled and treated with DVd regimen. Primary endpoints were tolerability and safety. Secondary endpoints were overall response rate (ORR), very good partial response...
Source: International Journal of Hematology - December 19, 2017 Category: Hematology Authors: Iida S, Ichinohe T, Shinagawa A, Suzuki K, Takezako N, Aoki M Tags: Int J Hematol Source Type: research

Prospective randomization of post-remission therapy comparing autologous peripheral blood stem cell transplantation versus high-dose cytarabine consolidation for acute myelogenous leukemia in first remission.
Abstract We prospectively compared outcomes of autologous stem cell transplantation (ASCT) versus high-dose cytarabine (HiDAC) consolidation as post-remission therapy for favorable- and intermediate-risk acute myelogenous leukemia (AML) in first complete remission (CR1). Two-hundred-forty patients under 65 years with AML-M1, M2, M4, or M5 subtypes were enrolled. After induction, 153 patients did not undergo randomization, while the remaining 87 who achieved CR1 were prospectively randomized to HiDAC (n = 45) or ASCT arm (n = 42). In the HiDAC arm, 43 patients completed three cycles of HiDA...
Source: International Journal of Hematology - December 14, 2017 Category: Hematology Authors: Miyamoto T, Nagafuji K, Fujisaki T, Uchida N, Matsue K, Henzan H, Ogawa R, Takase K, Aoki T, Hidaka M, Teshima T, Taniguchi S, Akashi K, Harada M, Japan Study Group for Cell Therapy and Transplantation (JSCT) Tags: Int J Hematol Source Type: research

Low-dose interleukin-2 as a modulator of Treg homeostasis after HSCT: current understanding and future perspectives.
Abstract CD4+CD25+Foxp3+ Treg is a functionally distinct subset of mature T cells with broad suppressive activity and has been shown to play an important role in the establishment of immune tolerance after HSCT. Altered cytokine environment in post-HSCT lymphopenia with a relative functional deficiency of IL-2 could hamper the reconstitution of Treg, leading to refractory GVHD. Based on the theory of low-dose IL-2 in which Treg can be selectively stimulated through the high-affinity IL-2 receptor, clinical studies have been conducted and demonstrated that low-dose IL-2 administration can restore Treg homeostasis a...
Source: International Journal of Hematology - December 12, 2017 Category: Hematology Authors: Matsuoka KI Tags: Int J Hematol Source Type: research

Sarcopenia after induction therapy in childhood acute lymphoblastic leukemia: its clinical significance.
In this study, patients with values less than the lowest quartile of MLI were classified into the sarcopenia group, and their basic and clinical factors were compared with those in the non-sarcopenia group. Muscle loss was observed in all patients after induction therapy, and severe adverse events during induction therapy were significantly more common in patients in the sarcopenia group. Furthermore, sarcopenia was found to be an independent prognostic factor for invasive fungal infection (IFI) that occurs after induction therapy. The evaluation of sarcopenia on CT images is easy and useful as a predictor of unfavorable e...
Source: International Journal of Hematology - December 12, 2017 Category: Hematology Authors: Suzuki D, Kobayashi R, Sano H, Hori D, Kobayashi K Tags: Int J Hematol Source Type: research

Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorders of childhood with fulminant leukocytosis and tumor lysis: a case report with autopsy findings.
We report an adolescent male with systemic EBV-positive T-LPD of childhood after primary EBV infection, resulting in a fatal clinical course within 9 days, along with autopsy findings. A 19-year-old male without an immunocompromised status presented with an acute onset of high fever, and was hospitalized for persistent fever, vomiting and diarrhea on the 5th day from onset. Laboratory data showed severe thrombocytopenia, increased ferritin level, liver dysfunction, disseminated intravascular coagulation, and anti-EBV-IgM positivity. Peripheral blood smears identified a number of atypical lymphocytes. Bone marrow ...
Source: International Journal of Hematology - December 7, 2017 Category: Hematology Authors: Wada S, Suzuki T, Kitazume K, Fujita A, Shimizu S Tags: Int J Hematol Source Type: research

Iron metabolism and related diseases: an overview.
PMID: 29209948 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - December 5, 2017 Category: Hematology Authors: Harigae H Tags: Int J Hematol Source Type: research

Correction to: Iron and infection.
Abstract The author would like to correct the error in the "Abstract" section of original publication as given below. PMID: 29197980 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - December 2, 2017 Category: Hematology Authors: Ganz T Tags: Int J Hematol Source Type: research

Modern iron replacement therapy: clinical and pathophysiological insights.
Abstract Iron deficiency, with or without anemia, is extremely frequent worldwide, representing a major public health problem. Iron replacement therapy dates back to the seventeenth century, and has progressed relatively slowly until recently. Both oral and intravenous traditional iron formulations are known to be far from ideal, mainly because of tolerability and safety issues, respectively. At the beginning of this century, the discovery of hepcidin/ferroportin axis has represented a turning point in the knowledge of the pathophysiology of iron metabolism disorders, ushering a new era. In the meantime, advances ...
Source: International Journal of Hematology - December 1, 2017 Category: Hematology Authors: Girelli D, Ugolini S, Busti F, Marchi G, Castagna A Tags: Int J Hematol Source Type: research

NK cell therapy after hematopoietic stem cell transplantation: can we improve anti-tumor effect?
Abstract After decades since the discovery of natural killer (NK) cells as potential effector cells fighting malignantly transformed and virally infected cells, little progress has been made in their clinical application. This yet unrealized therapeutic effect is presumably, at least in part, due to low numbers of functional NK cells that could be obtained from the peripheral blood relative to tumor burden. Our group hypothesized that a relatively small NK cell number to targeted malignant cells is the cause of a lack of clinical effect. We pursued obtaining large numbers of NK cells via ex vivo expansion using fe...
Source: International Journal of Hematology - December 1, 2017 Category: Hematology Authors: Van Elssen CHMJ, Ciurea SO Tags: Int J Hematol Source Type: research

Correction to: Effect of sertraline on complications and survival after hematopoietic stem-cell transplantation, a double-blind, placebo-controlled clinical study.
Abstract The correct name of the corresponding author should be ''Maryam Mehrpooya'', and not ''Mehrpooya Maryam'' as given in the original publication of the article. PMID: 29181700 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - November 27, 2017 Category: Hematology Authors: Tavakoli-Ardakani M, Kheshti R, Mehrpooya M Tags: Int J Hematol Source Type: research

Non-alcoholic steatohepatitis induced by induction chemotherapy for pediatric acute lymphoblastic leukemia.
PMID: 29181701 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - November 27, 2017 Category: Hematology Authors: Yamamoto M, Hori T, Igarashi K, Iesato K, Saito M, Miyanishi K, Kikuchi N, Fujita H, Tsutsumi H Tags: Int J Hematol Source Type: research

Three coexisting lymphomas in a single patient: composite lymphoma derived from a common germinal center B-cell precursor and unrelated discordant lymphoma.
Abstract Composite lymphoma (CL) is a rare disorder defined as the coexistence of two or more distinct lymphoma subtypes at a single anatomic site. Discordant lymphoma (DL), which is the simultaneous occurrence of two or more distinct lymphoma subtypes at different sites, is also rare. CL complicated with DL involving three distinct subtypes of lymphoma in the same patient is an extremely rare disease. Clonal relationships in CL and DL are commonly investigated by molecular analysis using mutational status with t(14;18)BCL2/IgH translocation and immunoglobulin heavy chain variable-region (IgVH) gene rearrangement....
Source: International Journal of Hematology - November 25, 2017 Category: Hematology Authors: Nishioka A, Ureshino H, Ando T, Kizuka H, Kusaba K, Sano H, Itamura H, Kubota Y, Kojima K, Ohshima K, Kimura S Tags: Int J Hematol Source Type: research

Iron overload in myelodysplastic syndromes (MDS).
Abstract Iron overload (IOL) starts to develop in MDS patients before they become transfusion-dependent because ineffective erythropoiesis suppresses hepcidin production in the liver and thus leads to unrestrained intestinal iron uptake. However, the most important cause of iron overload in MDS is chronic transfusion therapy. While transfusion dependency by itself is a negative prognostic factor reflecting poor bone marrow function, the ensuing transfusional iron overload has an additional dose-dependent negative impact on the survival of patients with lower risk MDS. Cardiac dysfunction appears to be important in...
Source: International Journal of Hematology - November 25, 2017 Category: Hematology Authors: Gattermann N Tags: Int J Hematol Source Type: research

ETV6-ABL1 fusion combined with monosomy 7 in childhood B-precursor acute lymphoblastic leukemia.
Abstract ETV6-ABL1 fusion is a rare but recurrent oncogenic lesion found in childhood B-cell precursor acute lymphoblastic leukemia (BCP-ALL), without an established chromosomal abnormality, and is associated with poor outcome. In ETV6-ABL1-positive cases, an in-frame fusion produced by a complex rearrangement results in constitutive chimeric tyrosine kinase activity. Monosomy 7 is also a rare and unfavorable chromosomal abnormality in childhood BCP-ALL. Here, we report a 14-year-old female BCP-ALL patient with ETV6-ABL1 fusion combined with monosomy 7. She was admitted to our hospital because of persistent fever....
Source: International Journal of Hematology - November 24, 2017 Category: Hematology Authors: Uemura S, Nishimura N, Hasegawa D, Shono A, Sakaguchi K, Matsumoto H, Nakamachi Y, Saegusa J, Yokoi T, Tahara T, Tamura A, Yamamoto N, Saito A, Kozaki A, Kishimoto K, Ishida T, Nino N, Takafuji S, Mori T, Iijima K, Kosaka Y Tags: Int J Hematol Source Type: research

Correction to: 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.
Discussion" and had no impact on the conclusion hence the bottom of the table is removed. PMID: 29149425 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - November 17, 2017 Category: Hematology Authors: Nogami K, Shima M, Fukutake K, Fujii T, Taki M, Matsushita T, Higasa S, Sato T, Sakai M, Arai M, Uchikawa H, Engl W, Abbuehl B, Konkle BA Tags: Int J Hematol Source Type: research

Iron and infection.
Abstract Iron is an essential trace metal for nearly all infectious microorganisms, and host defense mechanisms target this dependence to deprive microbes of iron. This review highlights mechanisms that are activated during infections to restrict iron on mucosal surfaces, in plasma and extracellular fluid, and within macrophages. Iron overload disorders, such as hereditary hemochromatosis or β-thalassemia, interfere with iron-restrictive host responses, and thereby cause increased susceptibility to infections with microbes that can exploit this vulnerability. Anemia of inflammation (formerly known as anemia o...
Source: International Journal of Hematology - November 16, 2017 Category: Hematology Authors: Ganz T Tags: Int J Hematol Source Type: research

Long-term outcome in patients treated at home during the pancytopenic phase after allogeneic haematopoietic stem cell transplantation.
ger M Abstract Patients undergoing allogeneic haematopoietic stem cell transplantation (HSCT) were given the option to be treated at home during the pancytopenic phase. Daily visits by a nurse and phone calls from a physician from the unit were part of the protocol. During almost two decades, 252 patients with haematological malignancies and non-malignant disorders were included. Median age was 47 (range 0-72) years. Myeloablative conditioning was given to 102 patients and reduced intensity to 150. Donors were matched unrelated (n = 160), HLA-identical siblings (n = 71), or HLA-mismatched ...
Source: International Journal of Hematology - November 15, 2017 Category: Hematology Authors: Ringdén O, Sadeghi B, Moretti G, Finnbogadottir S, Eriksson B, Mattsson J, Svahn BM, Remberger M Tags: Int J Hematol Source Type: research

Hypomethylating agents for treatment and prevention of relapse after allogeneic blood stem cell transplantation.
Abstract Despite the curative potential of allogeneic stem cell transplantation (allo-SCT) in patients with acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS), many patients will relapse. Until recently therapeutic options mainly consisted of palliative care, chemotherapy, donor lymphocyte infusions and second transplantation in selected cases. Still many patients either do not tolerate intensive therapies or do not achieve durable remissions and will finally succumb. Given this unmet medical need the hypomethylating agents (HMA), Azacitidine (Aza) and Decitabine (DAC) have been tested as salvage the...
Source: International Journal of Hematology - November 15, 2017 Category: Hematology Authors: Schroeder T, Rautenberg C, Haas R, Germing U, Kobbe G Tags: Int J Hematol Source Type: research

Iron metabolism in erythroid cells and patients with congenital sideroblastic anemia.
Abstract Sideroblastic anemias are anemic disorders characterized by the presence of ring sideroblasts in a patient's bone marrow. These disorders are typically divided into two types, congenital or acquired sideroblastic anemia. Recently, several genes were reported as responsible for congenital sideroblastic anemia; however, the relationship between the function of the gene products and ring sideroblasts is largely unclear. In this review article, we will focus on the iron metabolism in erythroid cells as well as in patients with congenital sideroblastic anemia. PMID: 29139060 [PubMed - as supplied by publi...
Source: International Journal of Hematology - November 14, 2017 Category: Hematology Authors: Furuyama K, Kaneko K Tags: Int J Hematol Source Type: research

The mechanisms of systemic iron homeostasis and etiology, diagnosis, and treatment of hereditary hemochromatosis.
Abstract Hereditary hemochromatosis (HH) is a group of genetic iron overload disorders that manifest with various symptoms, including hepatic dysfunction, diabetes, and cardiomyopathy. Classic HH type 1, which is common in Caucasians, is caused by bi-allelic mutations of HFE. Severe types of HH are caused by either bi-allelic mutations of HFE2 that encodes hemojuvelin (type 2A) or HAMP that encodes hepcidin (type 2B). HH type 3, which is of intermediate severity, is caused by bi-allelic mutations of TFR2 that encodes transferrin receptor 2. Mutations of SLC40A1 that encodes ferroportin, the only cellular iron...
Source: International Journal of Hematology - November 13, 2017 Category: Hematology Authors: Kawabata H Tags: Int J Hematol Source Type: research

A new prognostic score comprising lactate dehydrogenase, albumin and neutrophil to lymphocyte ratio to predict sensitivity to first-line chemotherapy in patients with peripheral T-cell lymphomas.
Abstract No standard therapy for peripheral T-cell lymphomas (PTCLs) has been established, and treatment outcomes are poor. Upfront stem cell transplantation has been investigated in several studies, some of which have reported promising outcomes. However, some patients maintain long-term remission after chemotherapy alone. It is thus important to predict sensitivity to first-line chemotherapy to optimize treatment strategies. In the present study, we retrospectively analyzed time to treatment failure (TTF) of first-line chemotherapy in 59 patients with PTCLs. On multivariate analysis for TTF, elevated lactate deh...
Source: International Journal of Hematology - November 4, 2017 Category: Hematology Authors: Kaito S, Kanemasa Y, Sasaki Y, Okuya T, Yamaguchi T, Funasaka C, Shimoyama T, Omuro Y, Hishima T, Maeda Y Tags: Int J Hematol Source Type: research

Notch1 expression is regulated at the post-transcriptional level by the 3' untranslated region in hematopoietic stem cell development.
Abstract In hematopoiesis, the expression of critical genes is regulated in a stage-specific manner to maintain normal hematopoiesis. Notch1 is an essential gene involved in the commitment and development of the T-cell lineage. However, the regulation of Notch1 in hematopoiesis is controversial, particularly at the level of hematopoietic stem cell (HSC). Here, we found that the expression of Notch1 is controlled at the post-transcriptional level in HSCs. HSCs express a considerable level of Notch1 mRNA, but its protein level is very low, suggesting a post-transcriptional suppression for Notch1. Using a retroviral ...
Source: International Journal of Hematology - November 2, 2017 Category: Hematology Authors: Mizuno S, Iino T, Ozawa H, Arinobu Y, Chong Y, Akashi K Tags: Int J Hematol Source Type: research

Long-term clinical remission maintained after cessation of zidovudine and interferon- α therapy in chronic adult T-cell leukemia/lymphoma.
Long-term clinical remission maintained after cessation of zidovudine and interferon-α therapy in chronic adult T-cell leukemia/lymphoma. Int J Hematol. 2017 Oct 31;: Authors: Cook LB, Rowan AG, Demontis MA, Sagawe S, Gillet NA, Melamed A, Greiller C, Witkover A, Bangham CRM, Taylor GP Abstract Globally, > 5-10 million people are estimated to be infected with Human T-lymphotropic virus type 1 (HTLV-1), of whom ~ 5% develop adult T-cell leukemia/lymphoma (ATL). Despite advances in chemotherapy, overall survival (OS) has not improved in the 35 years since HTLV-1 was fir...
Source: International Journal of Hematology - October 31, 2017 Category: Hematology Authors: Cook LB, Rowan AG, Demontis MA, Sagawe S, Gillet NA, Melamed A, Greiller C, Witkover A, Bangham CRM, Taylor GP Tags: Int J Hematol Source Type: research

Nilotinib vs. imatinib in Japanese patients with newly diagnosed chronic myeloid leukemia in chronic phase: long-term follow-up of the Japanese subgroup of the randomized ENESTnd trial.
Abstract In the ongoing, international, phase 3 study Evaluating Nilotinib Efficacy and Safety in Clinical Trials-Newly Diagnosed Patients (ENESTnd), nilotinib 300 and nilotinib 400 mg, both twice daily, are compared with imatinib 400 mg once daily for the treatment of newly diagnosed chronic myeloid leukemia in the chronic phase (CML-CP). Results for the overall population in ENESTnd (n = 846) showed that nilotinib resulted in higher response rates vs. imatinib and was well tolerated. Outcomes among Japanese patients in ENESTnd were specifically analyzed after 1 year of follow-up, and sho...
Source: International Journal of Hematology - October 26, 2017 Category: Hematology Authors: Nakamae H, Fukuda T, Nakaseko C, Kanda Y, Ohmine K, Ono T, Matsumura I, Matsuda A, Aoki M, Ito K, Shibayama H Tags: Int J Hematol Source Type: research

Enforced expression of MIR142, a target of chromosome translocation in human B-cell tumors, results in B-cell depletion.
Abstract MicroRNA142 (MIR142) is a target of chromosome translocations and mutations in human B-cell lymphomas. We analyzed an aggressive B-cell lymphoma carrying t(8;17)(q24;q22) and t(6;14)(p21;q32), and sought to explore the role(s) of MIR142 in lymphomagenesis. t(8;17)(q24;q22) involved MYC on 8q24 and pri-MIR142 on 17q22. MYC was activated by a promoter substitution by t(8;17)(q24;q22). t(8;17)(q24;q22) was an additional event after t(6;14) (p21;q32), which caused the over-expression of CCND3. Southern blot analyses revealed that the MIR142 locus was deleted from the affected allele, whereas Northern analyses...
Source: International Journal of Hematology - October 25, 2017 Category: Hematology Authors: Kuriyama K, Enomoto Y, Suzuki R, Watanuki J, Hosoi H, Yamashita Y, Murata S, Mushino T, Tamura S, Hanaoka N, Dyer M, Siebert R, Kiyonari H, Nakakuma H, Kitamura T, Sonoki T Tags: Int J Hematol Source Type: research

Gene analysis of inherited antithrombin deficiency and functional analysis of abnormal antithrombin protein (N87D).
Abstract Inherited antithrombin (AT) deficiency is one of the most clinically significant forms of congenital thrombophilia and follows an autosomal dominant mode of inheritance. We analyzed SERPINC1 in a patient who developed deep-vein thrombosis and low AT activity during pregnancy, and identified a novel missense mutation c.259A>G (p.Asn87Asp; N87D). Surprisingly, analysis of the parents' DNA showed that they did not possess this mutant, and thus, it may have been due to a de novo mutation. We also expressed this mutant AT protein in COS-1 cells and compared its intracellular localization and intracellular a...
Source: International Journal of Hematology - October 25, 2017 Category: Hematology Authors: Kamijima S, Sekiya A, Takata M, Nakano H, Murakami M, Nakazato T, Asakura H, Morishita E Tags: Int J Hematol Source Type: research

JSH guideline for tumors of hematopoietic and lymphoid tissues-leukemia: 3. Acute lymphoblastic leukemia/lymphoblastic lymphoma (ALL/LBL).
PMID: 29067593 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - October 24, 2017 Category: Hematology Authors: Takeuchi J, Kusumoto S, Akiyama H, Kanda Y, Izutsu K Tags: Int J Hematol Source Type: research

Genetic variation of Kr üppel-like factor 1 (KLF1) and fetal hemoglobin (HbF) levels in β(0)-thalassemia/HbE disease.
This study confirms that experimental down-regulation of KLF1 in β(0)-thalassemia/HbE-derived erythroblasts significantly increases HbF production (up to 52.3 ± 2.4%), albeit with slightly delayed erythroid terminal differentiation. KLF1 exome sequencing of 130 Thai β(0)-thalassemia/HbE patients without co-inheritance of α-thalassemia found six patients with KLF1 heterozygous mutations including rs2072596 (p.F182L; n = 5) and rs745347362 (p.P284L; n = 1) missense mutations. However, while these patients had high HbF levels (38.1 ± 7.5%), they were all as...
Source: International Journal of Hematology - October 24, 2017 Category: Hematology Authors: Khamphikham P, Sripichai O, Munkongdee T, Fucharoen S, Tongsima S, Smith DR Tags: Int J Hematol Source Type: research

High-dose chemotherapy and autologous peripheral blood stem cell transplantation with BCVAC regimen followed by maintenance chemotherapy for children with very high risk acute lymphoblastic leukemia.
Abstract Allogeneic hematopoietic stem cell transplantation (HSCT) is the recommended treatment for children with very high risk acute lymphoblastic leukemia (ALL), but it requires adequate institutional infrastructure, experience, and expertise, especially for alternative donor HSCT. We review our experience with high-dose chemotherapy (HDCT) and autologous peripheral blood stem cell transplantation (APBSCT), followed by post-APBSCT maintenance chemotherapy for children with very high risk ALL. Between August 1997 and November 2012, our institute was not successful with HLA-haploidentical HSCT. Thus, if pati...
Source: International Journal of Hematology - October 20, 2017 Category: Hematology Authors: Hong CR, Kang HJ, Park KD, Shin HY, Ahn HS Tags: Int J Hematol Source Type: research

Collision of metastatic malignant melanoma and acute myelogenous leukemia in the bone marrow.
PMID: 29043550 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - October 17, 2017 Category: Hematology Authors: Kobayashi N, Shimizu H, Hirato J, Handa H Tags: Int J Hematol Source Type: research

MAGI-1 expression is decreased in several types of human T-cell leukemia cell lines, including adult T-cell leukemia.
In this study we found that MAGI-1 expression is decreased in multiple (9 out of 15) human T-cell leukemia cell lines, including adult T-cell leukemia (ATL), T-cell acute lymphoblastic leukemia and chronic T-cell lymphocytic leukemia. The overexpression of MAGI-1 protein in a MAGI-1-low ATL cell line reduced cellular growth. While the overexpression of MAGI-1 protein in a MAGI-1-low ATL cell line reduced the Akt and MEK activities, the knockdown of MAGI-1 in a MAGI-1-high ATL cell line augmented the Akt and MEK activities. Collectively, the findings of the present study suggest that the decreased expression of MAGI-1 in hu...
Source: International Journal of Hematology - October 17, 2017 Category: Hematology Authors: Kozakai T, Takahashi M, Higuchi M, Hara T, Saito K, Tanaka Y, Masuko M, Takizawa J, Sone H, Fujii M Tags: Int J Hematol Source Type: research

Sudden blast phase in chronic myeloid leukemia developed during nilotinib therapy after major molecular response was achieved.
Abstract Sudden blast phase (SBP) is a rare event in which patients with chronic myeloid leukemia (CML) in complete cytogenetic response (CCyR) rapidly progress to the blast phase. Few patients on second-generation tyrosine kinase inhibitors (2nd TKIs) have been reported to develop SBP. Here, we report a 45-year-old man diagnosed with CML in the chronic phase in April 2008 and immediately started on imatinib therapy. He achieved CCyR 12 months after starting imatinib therapy. Imatinib was followed by treatment with the 2nd TKIs nilotinib and dasatinib from January 2011 to yield a better response. He achieved ...
Source: International Journal of Hematology - October 14, 2017 Category: Hematology Authors: Okada Y, Sato K, Kobayashi S, Nagao S, Takano K, Teramoto M, Tachi N, Kawamura T, Horiuchi T, Kato S, Saga R, Maekawa T, Yamamura T, Watanabe J, Kobayashi A, Kimura F Tags: Int J Hematol Source Type: research

Prognostic impact of minimal disseminated disease and immune response to NPM-ALK in Japanese children with ALK-positive anaplastic large cell lymphoma.
Abstract The prognostic impact of minimal disseminated disease (MDD) and anti-anaplastic lymphoma kinase (ALK) antibody titer in children with ALK-positive anaplastic large cell lymphoma (ALCL) was reported by an Italian/German group. Here, we examine their prognostic value in Japanese children with ALK-positive ALCL. We evaluated nucleophosmin (NPM)-ALK transcripts in 60 patients at diagnosis by RT-PCR and real-time PCR (qPCR). The antibody titer was assessed in 35 patients. Fifty-two percent were MDD positive by RT-PCR and 37% had more than 10 copies of NPM-ALK per 10(4) copies of ABL (10NCNs) by qPCR. Fifty-one...
Source: International Journal of Hematology - October 13, 2017 Category: Hematology Authors: Iijima-Yamashita Y, Mori T, Nakazawa A, Fukano R, Takimoto T, Tsurusawa M, Kobayashi R, Horibe K Tags: Int J Hematol Source Type: research

Prospective observational study on the first 51 cases of peripheral blood stem cell transplantation from unrelated donors in Japan.
Abstract The Japan Marrow Donor Program (JMDP) has facilitated unrelated peripheral blood stem cell transplantation (URPBSCT) since 2010. We conducted a prospective multicenter observational study to evaluate the feasibility of such transplantation. Between 2011 and 2014, 51 patients underwent URPBSCT from 8/8 allele-matched donors for hematological malignancies. The median age of the patients was 50 years; 21 had high-risk disease. Myeloablative conditioning regimens were used in 31 patients, and tacrolimus based graft-versus-host disease (GVHD) prophylaxis was used for all patients. The cumulative rate of e...
Source: International Journal of Hematology - October 13, 2017 Category: Hematology Authors: Goto T, Tanaka T, Sawa M, Ueda Y, Ago H, Chiba S, Kanamori H, Nishikawa A, Nougawa M, Ohashi K, Okumura H, Tanimoto M, Fukuda T, Kawashima N, Kato T, Okada K, Nagafuji K, Okamoto SI, Atsuta Y, Hino M, Tanaka J, Miyamura K Tags: Int J Hematol Source Type: research

Plasma cell myeloma mimicking classical Hodgkin lymphoma in the bone marrow.
PMID: 29027630 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - October 13, 2017 Category: Hematology Authors: Karner KH, Inamdar KV Tags: Int J Hematol Source Type: research

Fluconazole versus mould-active triazoles for primary antifungal prophylaxis in adult patients with acute lymphoblastic leukemia: clinical outcome and cost-effectiveness analysis.
This study evaluated the clinical and cost-effectiveness of prophylactic use of fluconazole versus mould-active triazoles (voriconazole and posaconazole) in adult patients with acute lymphoblastic leukemia (ALL). A decision analytical model was developed with inputs from a 7-year retrospective study (2009-2016) of 103 consecutive adult patients with ALL who received antifungal prophylaxis. Information on the administration of antifungal agents, clinical outcomes, and costs were collected. One-way sensitivity analyses and probabilistic sensitivity analysis were performed. The mould-active triazoles group was associated with...
Source: International Journal of Hematology - October 13, 2017 Category: Hematology Authors: Wang Y, Xing Y, Chen L, Meng T, Li Y, Xie J, Chen L, Dong Y, Dong W Tags: Int J Hematol Source Type: research

Dasatinib-associated reversible demyelinating peripheral polyneuropathy in a case of chronic myeloid leukemia.
We report a patient with CML who developed acute onset of DPN associated with dasatinib therapy. A 46-year-old Japanese woman was treated with dasatinib for 7 months after the diagnosis of CML and she achieved a major molecular response (MMR). However, dysphagia, hoarseness, and muscle weakness progressively developed over 2 weeks. Nerve conduction studies revealed extensive demyelinating changes. Dasatinib was discontinued and the patient received intravenous immunoglobulin (IVIg), resulting in resolution of the symptoms. However, 1 month after the re-initiation of dasatinib therapy, muscle weakness develop...
Source: International Journal of Hematology - October 13, 2017 Category: Hematology Authors: Ishida T, Akagawa N, Miyata T, Tominaga N, Iizuka T, Higashihara M, Suzuki T, Miyazaki K Tags: Int J Hematol Source Type: research

Prognostic value of genetic mutations in adolescent and young adults with acute myeloid leukemia.
Usui N, Kanamori H, Ito Y, Imai K, Suehiro Y, Kobayashi S, Kitamura K, Sakaida E, Ogawa S, Naoe T, Hayashi Y, Horibe K, Manabe A, Mizutani S, Adachi S, Kiyoi H Abstract Clinical outcomes and the genetic background of acute myeloid leukemia (AML) in adolescent and young adults (AYAs) are known to differ in younger children and older adults. To clarify the impact of genetic mutations on clinical outcomes of AYAs with AML, we analyzed data from the JPLSG AML-05 and JALSG AML201 studies. AYAs aged 15-39 years (n = 103) were included. FLT3-ITD, KIT, CEBPA, NRAS, KRAS, WT1, MLL-PTD, and NPM1 mutations wer...
Source: International Journal of Hematology - October 12, 2017 Category: Hematology Authors: Kuwatsuka Y, Tomizawa D, Kihara R, Nagata Y, Shiba N, Iijima-Yamashita Y, Shimada A, Deguchi T, Miyachi H, Tawa A, Taga T, Kinoshita A, Nakayama H, Kiyokawa N, Saito AM, Koh K, Goto H, Kosaka Y, Asou N, Ohtake S, Miyawaki S, Miyazaki Y, Sakura T, Ozawa Y, Tags: Int J Hematol Source Type: research

Anemia and hypogammaglobulinemia caused by M énétrier's disease.
Anemia and hypogammaglobulinemia caused by Ménétrier's disease. Int J Hematol. 2017 Oct 12;: Authors: Imataki O, Uchida S, Yokokura S, Uemura M, Kadowaki N PMID: 29027113 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - October 12, 2017 Category: Hematology Authors: Imataki O, Uchida S, Yokokura S, Uemura M, Kadowaki N Tags: Int J Hematol Source Type: research

Immature platelet fraction (IPF) as a predictive value for thrombopoietic recovery after allogeneic stem cell transplantation.
Abstract We consecutively examined the utility of measurements of percentage of immature platelet fraction (IPF%) and absolute IPF number (A-IPF) in predicting thrombopoietic recovery in 15 adult patients who underwent allogeneic hematopoietic stem cell transplantation (allo-SCT). Four patients were excluded from the evaluation due to insufficient data. Platelet count and IPF were measured by Sysmex XN-1000 (XN), a newer generation analyzer. First, we confirmed that platelet count measured by XN was more accurate than by XE-2100 (XE). IPF measurement was effective to predict the recovery in 7 of the 11 patients ex...
Source: International Journal of Hematology - October 12, 2017 Category: Hematology Authors: Sakuragi M, Hayashi S, Maruyama M, Kiyokawa T, Nagamine K, Fujita J, Maeda T, Kato H, Kashiwagi H, Kanakura Y, Tomiyama Y Tags: Int J Hematol Source Type: research

TAK1 inhibition ameliorates survival from graft-versus-host disease in an allogeneic murine marrow transplantation model.
Abstract Acute graft-versus-host disease (GVHD) is a major cause of morbidity and mortality in allogeneic hematopoietic cell transplantation (allo-HCT). Majority of the current immunosuppressive strategies targeting donor T cells to prevent or treat acute GVHD are only partially effective, and often require escalated immunosuppressive therapy. Recent studies have revealed that activation of antigen-presenting cells in the proinflammatory milieu is important for the priming and promotion of GVHD. This activation is mediated by innate immune signaling pathways, which therefore potentially represent new tar...
Source: International Journal of Hematology - October 12, 2017 Category: Hematology Authors: Kobayashi A, Kobayashi S, Miyai K, Osawa Y, Horiuchi T, Kato S, Maekawa T, Yamamura T, Watanabe J, Sato K, Tsuda H, Kimura F Tags: Int J Hematol Source Type: research

Correction to: JSH guideline for tumors of hematopoietic and lymphoid tissues-lukemia: 4. Chronic myelogenous leukemia (CML)/myeloproliferative neoplasms (MPN).
Abstract In the original publication of this article, the "Acknowledgements" was not included and it is given below for your reading. PMID: 29027146 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - October 12, 2017 Category: Hematology Authors: Usui N Tags: Int J Hematol Source Type: research