Comparison of HHV-6 DNA detection in plasma and whole blood in allogeneic hematopoietic stem cell transplant recipients: frequent false-positive results for active HHV-6 infection using whole blood samples.
Abstract In this prospective observational study, we compared the human herpesvirus-6 (HHV-6) DNA load in serially collected paired plasma and whole blood (WB) samples from allogeneic hematopoietic stem cell transplantation (HSCT) recipients. A total of 721 paired samples were collected from 68 recipients. The positive rate for HHV-6 DNA was 9.7 and 35.0% in plasma and WB samples, respectively (P 
Source: International Journal of Hematology - July 16, 2018 Category: Hematology Authors: Takano K, Ogata M, Kawano R, Satou T, Nashimoto Y, Shirao K Tags: Int J Hematol Source Type: research

The better outcomes of diffuse large B-cell lymphoma in adolescents and young adults.
PMID: 30008114 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - July 14, 2018 Category: Hematology Authors: Wang J, Yan F, Wang Y Tags: Int J Hematol Source Type: research

Molecular characterization of Hb H disease in southern Thailand.
Abstract Genotypes of 260 individuals with hemoglobin H (Hb H) disease originating from various provinces in southern Thailand were characterized by multiplex PCR (M-PCR) and reverse dot blot hybridization (RDB). M-PCR was used to amplify target fragments and then hybridized with allele-specific oligonucleotide (ASO) probes which were bound on a nylon membrane. A total of eight α-thalassemia (α-thal) mutations, which produced eight Hb H disease genotypes (α0-thal/α+-thal), were detected. The most common form of α0-thal was -SEA with a frequency of 99.23%. The other form (0.77%) of &al...
Source: International Journal of Hematology - July 13, 2018 Category: Hematology Authors: Nittayaboon K, Nopparatana C Tags: Int J Hematol Source Type: research

Invasive Scopulariopsis alboflavescens infection in patient with acute myeloid leukemia.
In this report, we described an adult woman with acute myeloid leukemia and who developed S. alboflavescens pneumonia. Liposomal amphotericin B and voriconazole combination therapy was unsuccessful and the patient died because of pneumonia. Scopulariopsis is highly resistant to available antifungal agents and almost invariably fatal. This case report should alert clinicians to the importance of listing Scopulariopsis as a pathogenic fungus in immunocompromised patients. PMID: 29987744 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - July 9, 2018 Category: Hematology Authors: Kurata K, Nishimura S, Ichikawa H, Sakai R, Mizutani Y, Takenaka K, Kakiuchi S, Miyata Y, Kitao A, Yakushijin K, Kawamoto S, Yamamoto K, Ito M, Matsuoka H, Tokimatsu I, Kamei K, Minami H Tags: Int J Hematol Source Type: research

Early/prefibrotic primary myelofibrosis in patients who were initially diagnosed with essential thrombocythemia.
Abstract A new entity, namely early/prefibrotic primary myelofibrosis (PMF), was introduced as a subtype of PMF in the 2016 revised World Health Organization (WHO) criteria for myeloproliferative neoplasms (MPN). It was diagnosed based on histopathological features of bone marrow (BM) biopsy specimens together with clinical parameters [leukocytosis, anemia, elevated lactate dehydrogenase (LDH) values, and splenomegaly]. The aim of this study was to evaluate the prevalence of early/prefibrotic PMF in patients who were previously diagnosed with ET, and to compare clinical features at diagnosis and outcomes between e...
Source: International Journal of Hematology - July 9, 2018 Category: Hematology Authors: Kamiunten A, Shide K, Kameda T, Ito M, Sekine M, Kubuki Y, Hidaka T, Akizuki K, Tahira Y, Toyama T, Kawano N, Marutsuka K, Maeda K, Takeuchi M, Kawano H, Sato S, Ishizaki J, Shimoda H, Yamashita K, Matsuoka H, Shimoda K Tags: Int J Hematol Source Type: research

Correction to: Effects of eculizumab treatment on quality of life in patients with paroxysmal nocturnal hemoglobinuria in Japan.
Abstract In the original publication of this article, Tables 2, 3 and 4 were published incorrectly. The corrected tables 2, 3 and 4 are given in the following pages. PMID: 29980910 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - July 6, 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

Quantification of bone-marrow plasma cell levels using various International Myeloma Working Group response criteria in patients with multiple myeloma.
We examined the association of residual bone-marrow plasma cells (PCs) and International Myeloma Working Group (IMWG) response assessment in light-chain-only multiple myeloma (LCMM) and intact immunoglobulin multiple myeloma (IIMM) using multicolour flow cytometry (MFC). We identify considerable differences in bone-marrow neoplastic PC levels between IIMM and LCMM for the same IMWG response categories. Furthermore, even in the same IMWG response category, residual neoplastic PC levels differed considerably over a logarithmic scale range However, normalization of the free light-chain ratio is associated with deeper response (
Source: International Journal of Hematology - July 5, 2018 Category: Hematology Authors: Narita K, Kobayashi H, Abe Y, Kitadate H, Takeuchi M, Matsue K Tags: Int J Hematol Source Type: research

Immuno-suppressive function of nucleus-transducible BAF57- ΔPH in T cell activation via degradation of endogenous BAF57.
In this study, a nucleus-transducible form of BAF57, absent the proline-rich and HMG domains (ntBAF57-ΔPH), was generated to interfere with the interaction between BAF57 and its binding protein, BAF155. ntBAF57-ΔPH was effectively delivered into mouse CD4+ T cells in a dose- and time-dependent manner, without cellular toxicity. Inhibition of T cell activation by ntBAF57-ΔPH was mediated by its disruption of the interaction between BAF155 and BAF57, leading to the degradation of endogenous BAF57 and BAF155. This phenomenon led to alterations in gene expression similar to those associated with Ciclosporin A...
Source: International Journal of Hematology - July 5, 2018 Category: Hematology Authors: Moon JS, Lee HJ, Ho CC, Shin JS, Ghosh S, Kim JH, Lee SK Tags: Int J Hematol Source Type: research

Clinical characteristics of pediatric patients with myeloid sarcoma without bone marrow involvement in Japan.
Abstract Myeloid sarcoma (MS) is a rare neoplastic condition that is often described in association with acute myeloid leukemia (AML). MS in childhood has received little attention, particularly in Japan. We carried out a nationwide retrospective analysis of Japanese children diagnosed with MS without bone marrow involvement. Inclusion criteria were diagnosis of MS at younger than 20 years of age between January 1, 2000 and December 31, 2013. There was a predominance of males (8:2), and the median age at MS diagnosis was 4 years. Sites of involvement varied and included skin (n = 3), head a...
Source: International Journal of Hematology - July 3, 2018 Category: Hematology Authors: Taga T, Imamura T, Nakashima K, Maeda N, Watanabe A, Miyajima Y, Sakaguchi S, Sano H, Hasegawa D, Kawasaki H, Adachi S, Takagi M, Koh K, Manabe A, Taki T, Ishida Y Tags: Int J Hematol Source Type: research

Re. Re. Dabigatran overdose: a case report of acute hepatitis. Extracorporeal treatment and review of the literature.
PMID: 29971603 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - July 3, 2018 Category: Hematology Authors: Porru M, Mameli A, Cianchetti ME, Ruberto F, Musu M, Barcellona D, Marongiu F Tags: Int J Hematol Source Type: research

Correction to: Atypical erythroblastosis in a patient with Diamond-Blackfan anemia who developed del(20q) myelodysplasia.
Abstract The corresponding author should be ''Masataka Ishimura'', and not ''Motoshi Sonoda'' as given in the original publication of the article. PMID: 29967977 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - July 2, 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

Obstetric outcomes and acceptance of alternative therapies to blood transfusion by Jehovah's Witnesses in Japan: a single-center study.
We examined the acceptance rates of blood transfusions, blood products, and autotransfusion types in patients who experienced postpartum hemorrhage (PPH), and investigated estimated hemorrhage volume at delivery and PPH treatments. Of the 84 JW patients, none accepted blood transfusion; however, 75 patients (89.3%) accepted blood products, 57 (67.9%) accepted autotransfusion using intraoperative cell salvage, and four (4.8%) refused all alternatives to blood transfusion. Furthermore, PPH > 1000 mL occurred in 18 of the 95 (18.9%) deliveries. Of these 18 patients, four (22.2%) required blood products a...
Source: International Journal of Hematology - June 29, 2018 Category: Hematology Authors: Tanaka M, Matsuzaki S, Endo M, Kakigano A, Mimura K, Takiuchi T, Miyake T, Tomimatsu T, Ueda Y, Kimura T Tags: Int J Hematol Source Type: research

Circulating tumor DNA dynamically predicts response and/or relapse in patients with hematological malignancies.
Abstract A growing body of evidence suggests that tumor-derived fragmentary DNA, known as circulating tumor DNA (ctDNA), has the potential to serve as a non-invasive biomarker for disease monitoring. However, in the setting of hematological malignancy, few published studies support the utility of ctDNA. We retrospectively investigated ctDNA levels of 17 patients with various hematological malignancies who had achieved remission after first-line therapy. We identified somatic driver mutations by next-generation sequencing, and designed droplet digital PCR assays for each mutation to measure ctDNA. Variant allele fr...
Source: International Journal of Hematology - June 29, 2018 Category: Hematology Authors: Nakamura S, Yokoyama K, Yusa N, Ogawa M, Takei T, Kobayashi A, Ito M, Shimizu E, Kasajima R, Wada Y, Yamaguchi R, Imoto S, Nagamura-Inoue T, Miyano S, Tojo A Tags: Int J Hematol Source Type: research

Extracorporeal photopheresis with TC-V in Japanese patients with steroid-resistant chronic graft-versus-host disease.
This study confirmed that ECP using the TC-V device was effective, with an acceptable toxicity profile. Further studies in larger cohorts are clearly warranted to determine its optimal use in Japanese patients with cGVHD. PMID: 29959747 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - June 29, 2018 Category: Hematology Authors: Okamoto S, Teshima T, Kosugi-Kanaya M, Kahata K, Kawashima N, Kato J, Mori T, Ozawa Y, Miyamura K Tags: Int J Hematol Source Type: research

Targeting transcription factors in acute myeloid leukemia.
Abstract Transcription factors recognize and bind to consensus sequence elements that are specific for each transcription factor, and the transcription factors then regulate downstream gene expression. In the bone marrow, transcription factors, such as C/EBPα, PU.1, and RUNX1, control essential genes to maintain the normal hematopoietic system. Dysregulation of transcription factors caused by gene mutations, chromosomal aberrations, or aberrant expression can lead to cancer, including acute myeloid leukemia. In the past, transcription factors were not considered "druggable" targets. However, a bett...
Source: International Journal of Hematology - June 28, 2018 Category: Hematology Authors: Takei H, Kobayashi SS Tags: Int J Hematol Source Type: research

Re-induction chemotherapy using FLAG-mitoxantrone for adult patients with relapsed acute leukemia: a single-center experience from United Arab Emirates.
Abstract We studied the outcome of 47 adult patients with relapsed acute leukaemia (AML = 25 and ALL = 22) treated with FLAG-mitoxantrone regimen. Median time to relapse was 10.7 months (range 1.9-27.7). Complete remission (CR2) was 60.1% which was significantly more frequent in ALL compared to AML (P = 0.049). WBC count  1 year were significantly predictor for CR2 in AML (P = 0.005 for both). Induction death was significantly higher in ALL compared to AML (P = 0.039). Median follow-up was 4.0 months (0.9-119.8) for ...
Source: International Journal of Hematology - June 27, 2018 Category: Hematology Authors: Hassan IB, Kristensen J, Al Qawasmeh K, Alam A Tags: Int J Hematol Source Type: research

Successful treatment of cytarabine-related neurotoxicity with corticosteroids, a case series.
We present two cases of cytarabine-related neurotoxicity treated with corticosteroids with complete resolution of symptoms. PMID: 29946854 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - June 26, 2018 Category: Hematology Authors: Dotson JL, Jamil MO Tags: Int J Hematol Source Type: research

Whole-exome analysis to detect congenital hemolytic anemia mimicking congenital dyserythropoietic anemia.
We examined 10 CDA patients via central review of bone marrow morphology and genetic analysis for congenital bone marrow failure syndromes. Sanger sequencing for CDAN1, SEC23B, and KLF1 was performed for all patients. We performed whole-exome sequencing in patients without mutation in these genes. Three patients carried pathogenic CDAN1 mutations, whereas no SEC23B mutations were identified in our cohort. WES unexpectedly identified gene mutations known to cause congenital hemolytic anemia in two patients: canonical G6PD p.Val394Leu mutation and SPTA1 p.Arg28His mutation. Comprehensive genetic analysis is warranted for mor...
Source: International Journal of Hematology - June 23, 2018 Category: Hematology Authors: Hamada M, Doisaki S, Okuno Y, Muramatsu H, Hama A, Kawashima N, Narita A, Nishio N, Yoshida K, Kanno H, Manabe A, Taga T, Takahashi Y, Miyano S, Ogawa S, Kojima S Tags: Int J Hematol Source Type: research

A unique clinical presentation of de novo acute promyelocytic leukemia as a myeloid sarcoma of the breast.
We describe the first case, to our knowledge, of de novo myeloid sarcoma of the breast confirmed as acute promyelocytic leukemia. Immunohistochemical analysis, flow cytometry, fluorescent in situ hybridization analysis and molecular analysis using RQ-PCR of tissue samples should be routine in determining the correct diagnosis in this setting. PMID: 29931624 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - June 21, 2018 Category: Hematology Authors: Oravcova I, Mikuskova E, Leitnerova M, Gyarfas J, Mlcakova A, Szepe P, Plank L, Demitrovicova L, Mikudova V, Cingelova S, Mego M, Drgona L Tags: Int J Hematol Source Type: research

Breakthrough infection of Geotrichum capitatum during empirical caspofungin therapy after umbilical cord blood transplantation.
Abstract We experienced a breakthrough fungal infection caused by Geotrichum capitatum during empirical therapy with caspofungin. A 68-year-old male patient with refractory acute lymphoblastic leukemia had received umbilical cord blood transplantation after two courses of induction therapy. Empirical therapy with caspofungin was initiated 5 days before transplantation. Tacrolimus was continuously infused to prevent graft-versus-host disease. A minidose of methotrexate was intravenously administered on days 1 and 3 post-transplantation, which was changed to prednisolone from day 7 due to severe mucositis. Duri...
Source: International Journal of Hematology - June 20, 2018 Category: Hematology Authors: Oya S, Muta T Tags: Int J Hematol Source Type: research

Impact of hospital length of stay on the risk of readmission and overall survival after allogeneic stem cell transplantation.
Abstract Patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT) are at high risk of readmission for complications. We sought to examine the association between HSCT hospital length of stay and the incidence of readmission and survival after discharge. We retrospectively reviewed the cases of 230 allo-HSCT recipients. The cumulative incidence of readmission with non-relapse transplant-related complications (including infections; acute and chronic GVHD; liver, lung, renal, or neurological complications; and haematological abnormalities) 2 years after the first discharge was 49.7% in ...
Source: International Journal of Hematology - June 19, 2018 Category: Hematology Authors: Seto A, Atsuta Y, Kawashima N, Ozawa Y, Miyamura K, Kiyoi H Tags: Int J Hematol Source Type: research

Severe acyclovir-resistant herpes simplex virus 1 infection following cord blood transplantation.
PMID: 29923124 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - June 19, 2018 Category: Hematology Authors: Shigemura T, Nakazawa Y, Yoshikawa T, Fujii H, Yamada S, Saijo M, Okuyama R Tags: Int J Hematol Source Type: research

Cell dynamics during differentiation therapy with all-trans retinoic acid in acute promyelocytic leukemia.
Abstract The introduction of all-trans retinoic acid (ATRA) has made acute promyelocytic leukemia (APL) a curable disease; however, early death prior to the completion of treatment remains a problem. In quantitative evaluation of response to ATRA treatment, lymphocytes must be excluded as they do not originally have t(15;17). We categorized peripheral blood leukocytes by nuclear morphology into polymorphonuclear cells (PMNs) comprising segmented granulocytes, and non-polymorphonuclear cells (NPMs) which includes lymphocytes, monocytes, band cells, and immature myeloid cells. We consecutively evaluated the ratio of...
Source: International Journal of Hematology - May 29, 2018 Category: Hematology Authors: Sato K, Sakai H, Saiki Y, Uchida A, Uemura Y, Yokoi S, Tsuruoka Y, Nishio Y, Matsunawa M, Suzuki Y, Isobe Y, Kato M, Tomita N, Inoue Y, Miura I Tags: Int J Hematol Source Type: research

A phase I, open-label, dose-escalation study of amrubicin in combination with lenalidomide and weekly dexamethasone in previously treated adults with relapsed or refractory multiple myeloma.
tke M Abstract This phase 1 study investigated the safety of the anthracycline amrubicin combined with lenalidomide and dexamethasone in adults with relapsed or refractory multiple myeloma. A standard 3 + 3 design was used. Patients received intravenous amrubicin 40-80 mg/m2 on day one, lenalidomide 15 mg orally on days 1-14, and dexamethasone 40 mg orally weekly on 21 day cycles. 14 patients were enrolled, and completed a median of three cycles. The maximum tolerated dose was not reached. One patient experienced dose limiting toxicity of dizziness and diarrhea. The most frequent ...
Source: International Journal of Hematology - May 25, 2018 Category: Hematology Authors: Dinner S, Dunn TJ, Price E, Coutré SE, Gotlib J, Berube C, Kaufman GP, Medeiros BC, Liedtke M Tags: Int J Hematol Source Type: research

Howell-Jolly bodies in systemic amyloidosis.
PMID: 29790005 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - May 22, 2018 Category: Hematology Authors: Ong SY, Ng HJ Tags: Int J Hematol Source Type: research

Copy number abnormality of acute lymphoblastic leukemia cell lines based on their genetic subtypes.
In this study, we performed genetic analysis of 83 B cell precursor acute lymphoblastic leukemia (B-ALL) cell lines. First, we performed multiplex ligation-dependent probe amplification analysis to identify copy number abnormalities (CNAs) in eight genes associated with B-ALL according to genetic subtype. In Ph+ B-ALL cell lines, the frequencies of IKZF1, CDKN2A/2B, BTG1, and PAX5 deletion were significantly higher than those in Ph- B-ALL cell lines. The frequency of CDKN2A/2B deletion in KMT2A rearranged cell lines was significantly lower than that in non-KMT2A rearranged cell lines. These findings suggest that CNAs are c...
Source: International Journal of Hematology - May 21, 2018 Category: Hematology Authors: Tomoyasu C, Imamura T, Tomii T, Yano M, Asai D, Goto H, Shimada A, Sanada M, Iwamoto S, Takita J, Minegishi M, Inukai T, Sugita K, Hosoi H Tags: Int J Hematol Source Type: research

Long-term complete remission following tandem autologous stem cell transplantation and consolidative radiotherapy for refractory mediastinal gray-zone lymphoma.
Abstract Mediastinal gray zone lymphoma (MGZL) is a provisional entity with intermediate features between classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma. Outcomes for patients with MGZL are reportedly poorer than those for patients with cHL or primary mediastinal large B-cell lymphoma. Additionally, no standard management guidelines for patients with MGZL are available, primarily due to its recent identification, rarity, and challenges in diagnosis. Although recent several studies have suggested dose-adjusted EPOCH-R (etoposide, doxorubicin, vincristine, cyclophosphamide, prednisolone, and ritu...
Source: International Journal of Hematology - May 21, 2018 Category: Hematology Authors: Takaishi K, Muto T, Mimura N, Takiguchi J, Nagao Y, Oshima-Hasegawa N, Tsukamoto S, Takeda Y, Mitsukawa S, Takeuchi M, Ohwada C, Ota S, Iseki T, Nakaseko C, Sakaida E Tags: Int J Hematol Source Type: research

IgM k multiple myeloma with monoclonal surface immunoglobulin expression.
PMID: 29779080 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - May 19, 2018 Category: Hematology Authors: Laribi K, Alani M, Lemaire P Tags: Int J Hematol Source Type: research

Activity of decitabine in pericardial myeloid sarcoma.
JFM PMID: 29779081 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - May 19, 2018 Category: Hematology Authors: Evers D, Bär BMAM, Gotthardt M, van der Velden WJFM Tags: Int J Hematol Source Type: research

Comprehensive molecular diagnosis of Epstein-Barr virus-associated lymphoproliferative diseases using next-generation sequencing.
Abstract Epstein-Barr virus (EBV) is associated with several life-threatening diseases, such as lymphoproliferative disease (LPD), particularly in immunocompromised hosts. Some categories of primary immunodeficiency diseases (PIDs) including X-linked lymphoproliferative syndrome (XLP), are characterized by susceptibility and vulnerability to EBV infection. The number of genetically defined PIDs is rapidly increasing, and clinical genetic testing plays an important role in establishing a definitive diagnosis. Whole-exome sequencing is performed for diagnosing rare genetic diseases, but is both expensive and time-co...
Source: International Journal of Hematology - May 18, 2018 Category: Hematology Authors: Ono S, Nakayama M, Kanegane H, Hoshino A, Shimodera S, Shibata H, Fujino H, Fujino T, Yunomae Y, Okano T, Yamashita M, Yasumi T, Izawa K, Takagi M, Imai K, Zhang K, Marsh R, Picard C, Latour S, Ohara O, Morio T Tags: Int J Hematol Source Type: research

CD3+/CD8+ T-cell density and tumoral PD-L1 predict survival irrespective of rituximab treatment in Chinese diffuse large B-cell lymphoma patients.
Abstract To investigate the prognostic value of tumor-infiltrating T-cell density and programmed cell death ligand-1 (PD-L1) expression in diffuse large B cell lymphoma (DLBCL). One-hundred-twenty-five Chinese DLBCL patients were enrolled in our study and provided samples; 76 of all cases were treated with rituximab (R). Tumor tissues were immunostained and analyzed for CD3+ and CD8+ tumor-infiltrating T-cell density, tumoral PD-L1, and microenvironmental PD-L1 (mPD-L1). The density of CD3 was rated as high in 33.6% cases, while 64.0% of DLBCLs were classified as high CD8 density. Of all cases, 16.8% were PD-L1+. ...
Source: International Journal of Hematology - May 10, 2018 Category: Hematology Authors: Shi Y, Deng L, Song Y, Lin D, Lai Y, Zhou L, Yang L, Li X Tags: Int J Hematol Source Type: research

Refinement of the Glasgow Prognostic Score as a pre-transplant risk assessment for allogeneic hematopoietic cell transplantation.
In this study, we refined the GPS by adding pre-transplant SF to improve its prognostic ability and enable better stratification; we call this revised index the HCT-specific revised Glasgow Prognostic Score (HCT-GPS). We observed that the HCT-GPS more accurately predicted NRM and early-term OS than the GPS. Moreover, the HCT-GPS provides an independent prognostic factor adjusted for the HCT-CI and disease status, and stratifies patients into four risk groups by OS and NRM. Thus, the HCT-GPS is a useful index for predicting early-term complications after allogeneic HCT in patients with hematopoietic diseases. PMID: 297...
Source: International Journal of Hematology - May 9, 2018 Category: Hematology Authors: Shibasaki Y, Suwabe T, Katagiri T, Tanaka T, Ushiki T, Fuse K, Sato N, Yano T, Kuroha T, Hashimoto S, Narita M, Furukawa T, Sone H, Masuko M Tags: Int J Hematol Source Type: research

Dysplastic features seen in a patient with acute myeloid leukemia harboring the KTM2A-TET1 fusion gene.
PMID: 29744807 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - May 9, 2018 Category: Hematology Authors: Kawai H, Matsushita H, Aoyama Y, Matsui K, Onizuka M, Ando K Tags: Int J Hematol Source Type: research

Safety of intra-articular transplantation of lentivirally transduced mesenchymal stromal cells for haemophilic arthropathy in a non-human primate.
Abstract Joint bleeding and resultant arthropathy are major determinants of quality of life in haemophilia patients. We previously developed a mesenchymal stromal cell (MSC)-based treatment approach for haemophilic arthropathy in a mouse model of haemophilia A. Here, we evaluated the long-term safety of intra-articular injection of lentivirally transduced autologous MSCs in non-human primates. Autologous bone-marrow-derived MSCs transduced with a lentiviral vector expressing coagulation factor VIII (FVIII) were injected into the left knee joint of cynomolgus monkeys. We first conducted codon optimization to increa...
Source: International Journal of Hematology - May 8, 2018 Category: Hematology Authors: Ohmori T, Mizukami H, Katakai Y, Kawai S, Nakamura H, Inoue M, Shu T, Sugimoto H, Sakata Y Tags: Int J Hematol Source Type: research

Association between OGG1 S326C CC genotype and elevated relapse risk in acute myeloid leukemia.
Abstract Recent studies have shown that tumors of relapsed acute myeloid leukemia (AML) present additional genetic mutations compared to the primary tumors. The base excision repair (BER) pathway corrects oxidatively damaged mutagenic bases and plays an important role in maintaining genetic stability. The purpose of the present study was to investigate the relationship between BER functional polymorphisms and AML relapse. We focused on five major polymorphisms: OGG1 S326C, MUTYH Q324H, APE1 D148E, XRCC1 R194W, and XRCC1 R399Q. Ninety-four adults with AML who achieved first complete remission were recruited. Genoty...
Source: International Journal of Hematology - May 8, 2018 Category: Hematology Authors: Gotoh N, Saitoh T, Takahashi N, Kasamatsu T, Minato Y, Lobna A, Oda T, Hoshino T, Sakura T, Shimizu H, Takizawa M, Handa H, Yokohama A, Tsukamoto N, Murakami H Tags: Int J Hematol Source Type: research

Guest editorial: Regulatory signaling in normal and abnormal hematopoiesis.
Abstract Stem cells are characterized by their unique ability to both self-renew and differentiate along multiple cellular lineages. Self-renewal and differentiation must be tightly controlled to ensure an appropriate stem cell pool in tissue over the lifetime of an organism. Elucidating the mechanisms controlling stem cell fate and maintenance remains a key challenge in stem cell biology. Hematopoietic stem cells (HSCs) are responsible for the lifelong production of multiple blood cell lineages. To remain functional, these cells must interact with a particular microenvironment, known as the stem cell niche. HSC n...
Source: International Journal of Hematology - May 4, 2018 Category: Hematology Authors: Arai F Tags: Int J Hematol Source Type: research

Regulation of unfolded protein response in hematopoietic stem cells.
Abstract Hematopoietic stem cells (HSCs) play a central role in hematopoietic regeneration, which has been demonstrated by thorough studies. In contrast, the cell cycle status and metabolic condition of HSCs define these cells as dormant. Recent studies have also revealed that protein metabolism is quite unique, as dormant HSCs have a lower protein synthesis rate and folding capacity. Under proliferative conditions, upon hematopoietic stress, HSCs need to deal with higher requirements of protein production to achieve fast and effective blood replenishment. In such cases, increased protein synthesis could exceed th...
Source: International Journal of Hematology - May 3, 2018 Category: Hematology Authors: Sigurdsson V, Miharada K Tags: Int J Hematol Source Type: research

Switching to nilotinib is associated with deeper molecular responses in chronic myeloid leukemia chronic phase with major molecular responses to imatinib: STAT1 trial in Japan.
Abstract The purpose of this clinical trial was to evaluate the efficacy of 2-year consolidation therapy using nilotinib (NIL) for achieving a molecular response (MR4.5, BCR-ABL1 IS  ≤ 0.0032% on the International Scale) in patients with chronic myeloid leukemia in the chronic phase (CML-CP) who had achieved a major molecular response (MMR, BCR-ABL1 IS  ≤ 0.1%) with imatinib (IM). We recruited 76 Japanese patients for this trial. Nilotinib 300 mg, twice daily, was administered for 2 years, and 74 patients were evaluated in the study. The median age was 55.0 years. The me...
Source: International Journal of Hematology - April 30, 2018 Category: Hematology Authors: Noguchi S, Nakaseko C, Nishiwaki K, Ogasawara H, Ohishi K, Tokuhira M, Noguchi M, Kimura H, Handa H, Mitani K, Miura M, Wakita H, Takahashi N, STAT study group Tags: Int J Hematol Source Type: research

Successful hemostatic management of major surgery for cervical spondylotic myelopathy in a patient with severe factor XI deficiency.
We report the case of a 72-year-old male with severe FXID who underwent a laminoplasty under sufficient, but minimal, FFP transfusion. Through detailed monitoring of activated partial thromboplastin time (APTT) and FXI activity at the perioperative period, we succeeded in hemostatic management of major surgery without significant blood loss and fluid overload. From the course of this case, we found that measuring FXI activity is superior to measuring APTT. Furthermore, we identified a novel homozygous mutation in F11 [NM_000128.3:c.1041C > A:p.(Tyr347*)] by whole exome sequencing. PMID: 29713955 [PubM...
Source: International Journal of Hematology - April 30, 2018 Category: Hematology Authors: Ogawa Y, Yanagisawa K, Uchiyama Y, Akashi N, Mieda T, Iizuka H, Inoue M, Shizuka R, Murakami M, Matsumoto N, Handa H Tags: Int J Hematol Source Type: research

Blastic plasmacytoid dendritic cell neoplasm arising from clonal hematopoiesis.
Abstract Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of myeloid neoplasm. Clonal evolution in the development of BPDCN remains to be elucidated. In the present study, we examined clonal evolution in a case of BPDCN by analyzing the distribution of gene mutations in tumor cells and non-tumor blood cells. The p.D1129fs and p.K1005fs TET2 mutations, p.P95H SRSF2 mutation, and p.L287fs NPM1 mutation were identified in a skin tumor at diagnosis and peripheral blood mononuclear cells at relapse. Notably, the p.D1129fs TET2 and p.L287fs NPM1 mutations were observed only in tumor cells, while th...
Source: International Journal of Hematology - April 28, 2018 Category: Hematology Authors: Suma S, Sakata-Yanagimoto M, Nguyen TB, Hattori K, Sato T, Noguchi M, Nannya Y, Ogawa S, Watanabe R, Fujimoto M, Nakamura N, Kusakabe M, Nishikii H, Kato T, Chiba S Tags: Int J Hematol Source Type: research

The localization of α-synuclein in the process of differentiation of human erythroid cells.
The localization of α-synuclein in the process of differentiation of human erythroid cells. Int J Hematol. 2018 Apr 24;: Authors: Araki K, Sugawara K, Hayakawa EH, Ubukawa K, Kobayashi I, Wakui H, Takahashi N, Sawada K, Mochizuki H, Nunomura W Abstract Although the neuronal protein α-synuclein (α-syn) is thought to play a central role in the pathogenesis of Parkinson's disease (PD), its physiological function remains unknown. It is known that α-syn is also abundantly expressed in erythrocytes. However, its role in erythrocytes is also unknown. In the present study, we investiga...
Source: International Journal of Hematology - April 24, 2018 Category: Hematology Authors: Araki K, Sugawara K, Hayakawa EH, Ubukawa K, Kobayashi I, Wakui H, Takahashi N, Sawada K, Mochizuki H, Nunomura W Tags: Int J Hematol Source Type: research

Severe graft-versus-host disease after allogeneic hematopoietic stem cell transplantation with residual mogamulizumab concentration.
PMID: 29671245 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - April 18, 2018 Category: Hematology Authors: Hosoi H, Mushino T, Nishikawa A, Hashimoto H, Murata S, Hatanaka K, Tamura S, Hanaoka N, Shimizu N, Sonoki T Tags: Int J Hematol Source Type: research

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