Effect of methotrexate dose in graft-versus-host disease prophylaxis after single-unit cord blood transplantation in adult acute myeloid leukemia
Int J Hematol. 2021 Feb 21. doi: 10.1007/s12185-021-03097-8. Online ahead of print.ABSTRACTTo investigate the association between methotrexate (MTX) dosage and engraftment, graft-versus-host disease (GVHD) incidence, and survival in umbilical cord blood transplantation (UCBT), we compared transplant outcomes after UCBT with various GVHD prophylaxis regimens, using registry data with additional data collection. Patients transplanted for acute myeloid leukemia with a calcineurin inhibitor (CNI) and either MTX or mycophenolate mofetil (MMF) combination were selected. In total, 888 single-unit UCBTs (MTX15-10-10, 415; MTX10-7-...
Source: International Journal of Hematology - February 21, 2021 Category: Hematology Authors: Seitaro Terakura Yachiyo Kuwatsuka Junichi Sugita Satoshi Takahashi Yukiyasu Ozawa Kazutaka Ozeki Satoshi Yoshioka Hirohisa Nakamae Toshiro Kawakita Masashi Sawa Satoshi Morishige Yuho Najima Yuna Katsuoka Emiko Sakaida Yasuji Kouzai Takafumi Kimura Tatsu Source Type: research

Eltrombopag restores erythropoiesis in refractory adult acquired pure red cell aplasia
Int J Hematol. 2021 Feb 21. doi: 10.1007/s12185-021-03100-2. Online ahead of print.ABSTRACTAcquired pure red cell aplasia is a rare condition characterized by normocytic normochromic anemia with severe reticulocytopenia. In refractory acquired pure red cell aplasia, the low response rate of immunosuppressive therapy also constitutes a challenge. We herein report the case of a 58-year-old male with refractory acquired pure red cell aplasia that was successfully treated by eltrombopag at a dose of 75 mg/day. After application of eltrombopag, the patient achieved complete remission and tolerated the treatment very well, with ...
Source: International Journal of Hematology - February 21, 2021 Category: Hematology Authors: Xiaoqing Liu Lang Cheng Yao He Run Zhang Ruinan Lu Jianfu Zhang Ming Hong Guangsheng He Jianyong Li Source Type: research

Correction to: Safety profile of brentuximab vedotin in Japanese patients with relapsed/refractory Hodgkin lymphoma or systemic anaplastic large cell lymphoma: a post-marketing surveillance study
Int J Hematol. 2021 Feb 18. doi: 10.1007/s12185-021-03091-0. Online ahead of print.NO ABSTRACTPMID:33604778 | DOI:10.1007/s12185-021-03091-0 (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 19, 2021 Category: Hematology Authors: Koji Izutsu Michinori Ogura Kensei Tobinai Kiyohiko Hatake Shigeru Sakamoto Masanori Nishimura Miyako Hoshino Source Type: research

Correction to: Safety profile of brentuximab vedotin in Japanese patients with relapsed/refractory Hodgkin lymphoma or systemic anaplastic large cell lymphoma: a post-marketing surveillance study.
PMID: 33604778 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 18, 2021 Category: Hematology Authors: Izutsu K, Ogura M, Tobinai K, Hatake K, Sakamoto S, Nishimura M, Hoshino M Tags: Int J Hematol Source Type: research

Real-world outcomes of AIDS-related Burkitt lymphoma: a retrospective study of 78 cases over a 10-year period.
Abstract We investigated the clinical characteristics and outcomes of acquired immunodeficiency syndrome-related Burkitt lymphoma (AIDS-BL). A single-center retrospective study was performed of 78 cases over a 10-year period. The baseline characteristics of enrolled patients included the following: median age, 46 years; median CD4+ T lymphocyte count, 156 cells/μL; advanced stage, 74.3%; > 1 extranodal site, 55.1%; international prognostic index (IPI) > 1, 85.9%; and elevated serum lactate dehydrogenase, 82.1%. The 1-year and 2-year overall survival (OS) rates were ...
Source: International Journal of Hematology - February 17, 2021 Category: Hematology Authors: Wang Z, Zhang R, Gong Z, Liu L, Shen Y, Chen J, Qi T, Song W, Tang Y, Sun J, Lin Y, Xu S, Yang J, Lu H Tags: Int J Hematol Source Type: research

Cord blood transplantation with a reduced-intensity conditioning regimen using fludarabine and melphalan for adult T-cell leukemia/lymphoma.
Abstract Adult T-cell leukemia/lymphoma (ATLL) is a peripheral T-cell lymphoma with a poor prognosis when treated with chemotherapy alone; therefore, allogeneic stem cell transplantation is a consideration. We attempted cord blood transplantation (CBT) using a reduced-intensity conditioning regimen without total body irradiation (non-TBI-RIC) to allow for the best possible timing of transplantation and improve survival outcomes, particularly in older patients. Forty-eight patients (27 male, 21 female) underwent CBT using fludarabine (Flu) 125 mg/m2 and melphalan (Mel) 140 mg/m2 as pre-transplant conditio...
Source: International Journal of Hematology - February 16, 2021 Category: Hematology Authors: Nakano N, Takatsuka Y, Kubota A, Tokunaga M, Miyazono T, Tabuchi T, Odawara J, Tokunaga M, Makino T, Takeuchi S, Ito Y, Utsunomiya A Tags: Int J Hematol Source Type: research

EBV-positive diffuse large B-cell lymphoma, not otherwise specified masking Richter syndrome.
PMID: 33594655 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 16, 2021 Category: Hematology Authors: Lu S, Zhao S Tags: Int J Hematol Source Type: research

Quality of life and patient-reported outcomes in chronic severe neutropenia conditions.
We present the largest study to date of QOL in those with chronic severe neutropenia conditions with further evaluation of patient provider satisfaction and patient-reported outcome measures. Subjects completed electronic surveys assessing QOL, PROs, and patient provider satisfaction. There is a significantly negative impact of a chronic severe neutropenia disorder on QOL, fatigue, physical function, cognitive function and pain in adult patients when compared to controls. Children with a chronic neutropenia condition had comparable QOL to controls, but reported fewer depressive symptoms, improved mobility, and stronger sel...
Source: International Journal of Hematology - February 15, 2021 Category: Hematology Authors: Michniacki TF, Merz LE, McCaffery H, Connelly JA, Walkovich K Tags: Int J Hematol Source Type: research

Correction to: Impact of cGVHD on socioeconomic outcomes in survivors with pediatric hematopoietic stem cell transplant in Japan: a cross-sectional observational study.
PMID: 33576915 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 12, 2021 Category: Hematology Authors: Soejima T, Shiohara M, Ishida Y, Inoue M, Hayakawa A, Sato A, Kamibeppu K, Atsuta Y, Yamashita T Tags: Int J Hematol Source Type: research

Down syndrome with neonatal alloimmune thrombocytopenia due to anti-HLA A31 and B61 antibodies.
Abstract Neonatal alloimmune thrombocytopenia (NAIT) arises from fetomaternal platelet incompatibility that results in transplacental passage of maternal antibodies mostly against fetal human platelet antigens (HPA), whereas NAIT due to anti-human leukocyte antigen (HLA) antibodies is extremely rare. Here, we report a case of Down syndrome (DS) with NAIT that was attributed to HLA antibodies. A boy with DS was delivered at 36 weeks' gestation. His platelet count declined to 13.0 × 109/L, suggestive of NAIT rather than other conditions, including transient abnormal myelopoiesis. Random plat...
Source: International Journal of Hematology - February 11, 2021 Category: Hematology Authors: Shima E, Go H, Maeda H, Ogasawara K, Imamura T, Sasaki M, Koh Y, Nollet KE, Ikeda K, Ohto H, Hosoya M Tags: Int J Hematol Source Type: research

Minimal residual disease (MRD) positivity at allogeneic hematopoietic cell transplantation, not the quantity of MRD, is a risk factor for relapse of Philadelphia chromosome-positive acute lymphoblastic leukemia.
Abstract Minimal residual disease (MRD) monitoring by quantitative real-time reverse transcription PCR (qRT-PCR) is the standard of care in Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph-positive ALL). We evaluated the impact of MRD status at hematopoietic cell transplantation (HCT) on relapse, as measured by a unified protocol at a central laboratory. Only patients with Ph-positive ALL who had minor transcripts (e1a2) and who underwent allogeneic HCT in first complete remission between 2008 and 2017 were included. First, patients with negative-MRD (n = 196) and positive-MRD (n&thi...
Source: International Journal of Hematology - February 11, 2021 Category: Hematology Authors: Akahoshi Y, Arai Y, Nishiwaki S, Mizuta S, Marumo A, Uchida N, Kanda Y, Sakai H, Takada S, Fukuda T, Fujisawa S, Ashida T, Tanaka J, Atsuta Y, Kako S Tags: Int J Hematol Source Type: research

Clot waveform analysis for perioperative hemostatic monitoring of arthroscopic synovectomy in a pediatric patient with hemophilia A and inhibitor receiving emicizumab prophylaxis.
We describe a 14-year-old boy with HA-I receiving emicizumab prophylaxis who experienced arthroscopic synovectomy. He was treated with a bolus infusion of recombinant factor VIIa (rFVIIa; 80 μg/kg) immediately before surgery, and treatment continued at the same dose every 3 h on day 1, every 4 h on day 2, and every 6 h on day 3. Treatment with rFVIIa was discontinued on day 4. No perioperative bleeding or thrombotic events were observed. Coagulation potential throughout the perioperative period was retrospectively assessed with an easy-to-use clot waveform analysis (CWA). Measurements from CWA return...
Source: International Journal of Hematology - February 10, 2021 Category: Hematology Authors: Mizumachi K, Tsumura Y, Nakajima Y, Koh K, Nogami K Tags: Int J Hematol Source Type: research

Salvage therapy with 2-chlorodeoxyadenosine for refractory and relapsed pediatric Langerhans cell histiocytosis: an updated nationwide survey in Japan.
PMID: 33564966 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 9, 2021 Category: Hematology Authors: Taniguchi M, Sakamoto K, Shioda Y, Doi T, Kudo K, Fujino H, Kudo K, Morimoto A Tags: Int J Hematol Source Type: research

Flow cytometry analysis of peripheral Tregs in patients with multiple myeloma under lenalidomide maintenance.
PMID: 33559085 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 8, 2021 Category: Hematology Authors: Nozaki K, Fujioka Y, Sugiyama D, Ishikawa J, Iida M, Shibata M, Kosugi S, Nishikawa H, Shibayama H Tags: Int J Hematol Source Type: research

Diagnosis and treatment of disseminated intravascular coagulation in COVID-19 patients: a scoping review.
CONCLUSION: Studies on DIC diagnostic criteria and anticoagulants were limited to the ISTH or SIC scores and heparinoids, particularly LMWH. Further studies are needed to compare these with other available DIC scoring systems and anticoagulants. PMID: 33550533 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - February 7, 2021 Category: Hematology Authors: Mori H, Ohkawara H, Togawa R, Rikimaru M, Shibata Y, Ikezoe T Tags: Int J Hematol Source Type: research

Cytokine levels following allogeneic hematopoietic cell transplantation: a match-pair analysis of home care versus hospital care.
n BM, Sadeghi B Abstract Following allogeneic hematopoietic cell transplantation (HCT), patients living near the hospital were treated at home instead of in isolation in the hospital. We analyzed cytokines using Luminex assays for the first 3 weeks after HCT and compared patients treated at home (n = 42) with matched patients isolated in the hospital (n = 37). In the multivariate analysis, patients treated at home had decreased GM-CSF, IFN-γ (p 
Source: International Journal of Hematology - February 5, 2021 Category: Hematology Authors: Ringdén O, Remberger M, Törlén J, Finnbogadottir S, Svahn BM, Sadeghi B Tags: Int J Hematol Source Type: research

Biopsy remains indispensable for evaluating bone marrow involvement in DLBCL patients despite the use of positron emission tomography.
This study examined whether staging by PET/CT is sufficient. Participants with untreated DLBCL assessed using both PET/CT and BMB were included. Patients received independent diagnostic assessments from a radiologist and a hematopathologist. Both hematoxylin-eosin staining and CD20 immunostaining were performed to determine the bone marrow involvement in BMB. A total of 84 patients were included. The number of patients with positive bone marrow involvement identified by PET/CT and BMB was 16 (19%) and 22 (26%), respectively. Eight (10%) patients showed positive results in both tests. When considering BMB as a reference, PE...
Source: International Journal of Hematology - January 30, 2021 Category: Hematology Authors: Saiki Y, Tomita N, Uchida A, Uemura Y, Suzuki Y, Hirakawa T, Kato M, Hoshikawa M, Kawano T, Nakamura N, Miura I, Arai A Tags: Int J Hematol Source Type: research

Characteristics and transplant outcome of myeloid sarcoma: a single-institute study.
Abstract We performed a retrospective study describing the characteristics of myeloid sarcoma (MS) and evaluated the outcome of hematopoietic stem cell transplantation (HSCT) in patients with MS. There were 27 patients with de novo isolated MS, 34 with de novo leukemic MS and 13 with secondary leukemic MS in our study. Sixty-three patients received induction chemotherapy. Following induction therapy, 35 patients underwent HSCT, including 10 autogenous HSCT (auto-HSCT) and 25 allogeneic HSCT (allo-HSCT) cases. Compared with intensive chemotherapy only as consolidation treatment, HSCT (auto-/allo-HSCT) significantly...
Source: International Journal of Hematology - January 28, 2021 Category: Hematology Authors: Shan M, Lu Y, Yang M, Wang P, Lu S, Zhang L, Qiu H, Chen S, Xu Y, Zhang X, Wu D Tags: Int J Hematol Source Type: research

A simple method to distinguish residual elotuzumab from monoclonal paraprotein in immunofixation assays for multiple myeloma patients.
Abstract Negative immunofixation electrophoresis (IFE) of serum and/or urine is a diagnostic marker for determining a complete response (CR) after immunotherapy for multiple myeloma (MM). However, residual therapeutic antibodies such as elotuzumab (IgG-κ), can compromise IFE evaluation when the affected immunoglobulins belong to the same IgG-κ subclass. We thus sought to develop a simple and rapid method to treat patient serum before IFE to distinguish the residual elotuzumab. Serum samples from patients receiving elotuzumab were treated with a predetermined amount of soluble signaling lymphocyte activ...
Source: International Journal of Hematology - January 28, 2021 Category: Hematology Authors: Chen S, Kiguchi T, Nagata Y, Tamai Y, Ikeda T, Kajiya R, Ono T, Sugiyama D, Nishikawa H, Akatsuka Y Tags: Int J Hematol Source Type: research

WT1-specific CD8  + cytotoxic T cells with the capacity for antigen-specific expansion accumulate in the bone marrow in MDS.
WT1-specific CD8 + cytotoxic T cells with the capacity for antigen-specific expansion accumulate in the bone marrow in MDS. Int J Hematol. 2021 Jan 27;: Authors: Suwabe T, Shibasaki Y, Sato H, Tamura S, Katagiri T, Nemoto H, Kasami T, Kozakai T, Nanba A, Kitajima T, Fuse K, Ushiki T, Sone H, Narita M, Masuko M Abstract Wilms' tumor 1 (WT1) is a tumor-associated antigen and immunotherapy target in myelodysplastic syndrome (MDS). Further information is needed on the characteristics of WT1-specific CD8 + T cells to develop immunotherapeutic strategies for MDS. To clarify the f...
Source: International Journal of Hematology - January 27, 2021 Category: Hematology Authors: Suwabe T, Shibasaki Y, Sato H, Tamura S, Katagiri T, Nemoto H, Kasami T, Kozakai T, Nanba A, Kitajima T, Fuse K, Ushiki T, Sone H, Narita M, Masuko M Tags: Int J Hematol Source Type: research

Monitoring minimal/measurable residual disease in B-cell acute lymphoblastic leukemia by flow cytometry during targeted therapy.
Abstract B-cell acute lymphoblastic leukemia (B-ALL) is a hematologic malignancy of B-type lymphoid precursor cells. Minimal/measurable residual disease (MRD) is an important prognostic factor for B-ALL relapse. Traditional flow cytometry detection mainly relies on CD19-based gating strategies. However, relapse of CD19-negative B-ALL frequently occurs in patients who receive cellular and targeted therapy. This review will summarize the technical aspects of standard MRD assessment in B-ALL by flow cytometry, and then discuss the challenges of MRD strategies to deal with the scenario of CD19 negative or dim B-ALL re...
Source: International Journal of Hematology - January 27, 2021 Category: Hematology Authors: Liu Z, Li Y, Shi C Tags: Int J Hematol Source Type: research

Latest trends in the incidence of Helicobacter pylori-uninfected gastric mucosa-associated lymphoid tissue lymphoma at the Cancer Institute Hospital, Japan.
PMID: 33486669 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 24, 2021 Category: Hematology Authors: Ishioka M, Hirasawa T, Mishima Y Tags: Int J Hematol Source Type: research

Correction to: FLEND (nelarabine, fludarabine, and etoposide) for relapsed T-cell acute lymphoblastic leukemia in children: a report from Japan Children's Cancer Group.
PMID: 33484418 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 23, 2021 Category: Hematology Authors: Kumamoto T, Goto H, Ogawa C, Hori T, Deguchi T, Araki T, Saito AM, Manabe A, Horibe K, Toyoda H Tags: Int J Hematol Source Type: research

Successful upfront cord blood transplantation for plasma cell leukemia in the first complete response after daratumumab therapy.
Abstract Plasma cell leukemia (PCL) is a rare and aggressive disease with a poor prognosis. Autologous or allogeneic stem cell transplantation (ASCT or allo-SCT) with intensive chemotherapy is performed for PCL, but their efficacy is still controversial. The efficacy of novel agents such as daratumumab for PCL is also unclear. Here, we report a case of PCL treated successfully with daratumumab and upfront cord blood transplantation (CBT) in the first complete response (CR). A 58-year-old man was diagnosed with PCL based on elevated abnormal plasma cells and IgD levels. After two cycles of bortezomib, lenalidomide,...
Source: International Journal of Hematology - January 22, 2021 Category: Hematology Authors: Ueno T, Sugio Y, Ohta T, Uehara Y, Ohno Y Tags: Int J Hematol Source Type: research

Prognostic impact of 18F-FDG PET/CT in patients with multiple myeloma presenting with renal impairment.
This study investigated the prognostic impact of 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) in patients with MM with RI at diagnosis. The records of 209 patients with MM between June 2011 and November 2018 were retrospectively analyzed. PET/CT positivity was defined as the presence of more than three focal lesions or the presence of extramedullary disease. Of 209 patients, 90 (43.1%) had RI and showed similar survival outcomes to patients who had normal renal function. In total, 113 patients (54.0%) were PET/CT-positive, and 46.6% of patients with RI were PET/CT-positive at b...
Source: International Journal of Hematology - January 21, 2021 Category: Hematology Authors: Ahn SY, Park HK, Moon JH, Baek DW, Cho HJ, Sohn SK, Kang SR, Min JJ, Bom HS, Hong CM, Jeong SY, Song GY, Yang DH, Ahn JS, Kim HJ, Jung SH, Lee JJ Tags: Int J Hematol Source Type: research

Ethnic differences in thromboprophylaxis for COVID-19 patients: should they be considered?
Abstract Thromboembolic events contribute to morbidity and mortality in coronavirus disease 2019 (COVID-19). As a result, thromboprophylaxis using low-molecular-weight heparin (LMWH) is universally recommended for hospitalized patients based on multiple guidelines. However, ethnic differences with respect to thrombogenicity have been reported and the incidence of thromboembolic events is considered to be lower in the Asian population. Despite the importance of thromboprophylaxis, bleeding is also a side effect that should be considered. We examine the data relating to potential ethnic differences in thrombosis and...
Source: International Journal of Hematology - January 20, 2021 Category: Hematology Authors: Iba T, Connors JM, Spyropoulos AC, Wada H, Levy JH Tags: Int J Hematol Source Type: research

Allogeneic hematopoietic stem cell transplantation for adult T-cell leukemia/lymphoma with HTLV-1-associated myelopathy.
Abstract Allogeneic hematopoietic stem cell transplantation (allo-HSCT) can be curative for adult T-cell leukemia/lymphoma (ATL), but comorbidities increase transplant-related mortality. Here we report the outcome of allo-HSCT in a patient with ATL with human T-cell leukemia virus type I (HTLV-1)-associated myelopathy-tropical spastic paraparesis (HAM/TSP). A 48-year-old man was diagnosed with HAM/TSP and started prednisolone therapy. Ten years later, he developed lymphoma-type ATL. At the diagnosis of ATL, Osame's Motor Disability Score (OMDS) was 4. When prednisolone was gradually tapered and stopped following c...
Source: International Journal of Hematology - January 10, 2021 Category: Hematology Authors: Sakamoto H, Itonaga H, Sawayama Y, Kojima A, Chiwata M, Fujioka M, Kitanosono H, Horai M, Miyazaki T, Shiraishi H, Imaizumi Y, Yoshida S, Hata T, Yamano Y, Miyazaki Y Tags: Int J Hematol Source Type: research

Comparative evaluation of reagents for measuring protein S activity: possibility of harmonization.
This study aimed to confirm the possibility of harmonization of these three reagents to establish a universal standard for PS activity in Japanese populations. Commercial normal plasma and plasma samples obtained from healthy individuals and healthy pregnant women were tested at three facilities using three reagents for measuring PS: STA-Staclot Protein S (STA-PS), HemosIL Protein S (Clotting) (IL-PS), and a total PS assay (SNT-PS). The within-run precision of each reagent was good, as each had a coefficient of variation of ≤ 3.8%. The dilution linearity for each reagent was also good. The correlation coef...
Source: International Journal of Hematology - January 8, 2021 Category: Hematology Authors: Ieko M, Hotta T, Watanabe K, Adachi T, Takeuchi S, Naito S, Yoshida M, Ohmura K, Takahashi N, Morishita E, Tsuda H, Kang D Tags: Int J Hematol Source Type: research

Bone marrow involvement by CD1a-negative langerhans cell histiocytosis with associated hemophagocytosis.
PMID: 33404985 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 6, 2021 Category: Hematology Authors: Al Moosawi M, Marcon K Tags: Int J Hematol Source Type: research

Glycosylated ferritin as an improved marker for post-transfusion iron overload.
Abstract Red blood cell (RBC) transfusion is an effective therapy for anemia, but repeated transfusions may cause iron overload-related damage to various organs. Iron chelation therapy, now widely available for patients who have received transfusions, is expected to reduce organ damage even in patients who received many transfusions. Therefore, determining when to start iron chelation therapy is important. In guidelines for iron chelation therapy, the serum ferritin level has been widely accepted as a practical marker for estimating iron overload. However, guidelines recommend multiple measurements of serum ferrit...
Source: International Journal of Hematology - January 5, 2021 Category: Hematology Authors: Ishihara A, Yamauchi T, Ikeda K, Fukuyoshi Y, Yokoyama T, Yonemura Y, Uchiba M, Matsui H Tags: Int J Hematol Source Type: research

Extramedullary hematopoiesis of the cranial dura.
PMID: 33400142 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 5, 2021 Category: Hematology Authors: Suto K, Sugita J, Hashimoto D, Kameda H, Mitsuhashi T, Teshima T Tags: Int J Hematol Source Type: research

Development of Philadelphia chromosome-negative acute myeloid leukemia with IDH2 and NPM1 mutations in a patient with chronic myeloid leukemia who showed a major molecular response to tyrosine kinase inhibitor therapy.
Abstract Tyrosine kinase inhibitors (TKIs) are standard therapies for chronic myeloid leukemia (CML) that can eradicate Ph-positive leukemic cells. However, disease control is not achievable in a minority of cases, most commonly due to evolution of TKI-resistant clones. There have also been rare cases of emergence of Ph-negative clones with other cytogenetic abnormalities, and, less commonly, development of Ph-negative acute myeloid leukemia (AML), whose molecular pathogenesis is largely unknown. Here we report molecular features of a patient with Ph + CML who developed Ph-negative AML after showing ...
Source: International Journal of Hematology - January 5, 2021 Category: Hematology Authors: Nakamura F, Arai H, Nannya Y, Ichikawa M, Furuichi S, Nagasawa F, Takahashi W, Handa T, Nakamura Y, Tanaka H, Nakamura Y, Sasaki K, Miyano S, Ogawa S, Mitani K Tags: Int J Hematol Source Type: research

Correction to: Direct oral anticoagulants in patients with severe inherited thrombophilia: a single-center cohort study.
PMID: 33400144 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 5, 2021 Category: Hematology Authors: Zuk J, Papuga-Szela E, Zareba L, Undas A Tags: Int J Hematol Source Type: research

Pediatric blastic plasmacytoid dendritic cell neoplasm: report of four cases and review of literature.
Abstract Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematological malignancy with poor outcome. Four children with BPDCN treated at our hospital were enrolled. All the four cases presented with cutaneous lesions. Bone marrow and central nervous system was involved in 50% and 25% of patients, respectively. The whole exome sequencing analysis revealed that KMT2 family genes were the most frequently mutated (4/4, 100%), followed by IKZF2 (2/4, 50%). The point mutation p.D348N was found in three patients and one patient had p.C394Y mutation in the KMT2C gene. Translocation of KMT2A-M...
Source: International Journal of Hematology - January 4, 2021 Category: Hematology Authors: Liao C, Hu NX, Song H, Zhang JY, Shen DY, Xu XJ, Tang YM Tags: Int J Hematol Source Type: research

Clinical features of 35 Down syndrome patients with transient abnormal myelopoiesis at a single institution.
Abstract Transient abnormal myelopoiesis (TAM) is a unique clonal myeloproliferation characterized by immature megakaryoblasts that occurs in 5-10% of neonates with Down syndrome (DS). Although TAM regresses spontaneously in most patients, approximately 20% of TAM cases result in early death, and approximately 20% of survivors develop acute megakaryoblastic leukemia (AMKL). We retrospectively reviewed records of 35 DS patients with TAM to determine the correlation between clinical characteristics and blast percentage. Thirteen of the 35 patients were classified as low blast percentage TAM (LBP-TAM), defined as TAM...
Source: International Journal of Hematology - January 4, 2021 Category: Hematology Authors: Yamato G, Park MJ, Sotomatsu M, Kaburagi T, Maruyama K, Kobayashi T, Nishi A, Sameshima K, Ohki K, Hayashi Y Tags: Int J Hematol Source Type: research

The eyes have it: CNS leukemia presenting as optic neuritis.
PMID: 33394337 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 4, 2021 Category: Hematology Authors: Quann KA, Redner RL Tags: Int J Hematol Source Type: research

Histidine decarboxylase deficiency inhibits NBP-induced extramedullary hematopoiesis by modifying bone marrow and spleen microenvironments.
Abstract Histidine decarboxylase (HDC), a histamine synthase, is expressed in various hematopoietic cells and is induced by hematopoietic cytokines such as granulocyte colony-stimulating factor (G-CSF). We previously showed that nitrogen-containing bisphosphonate (NBP)-treatment induces extramedullary hematopoiesis via G-CSF stimulation. However, the function of HDC in NBP-induced medullary and extramedullary hematopoiesis remains unclear. Here, we investigated changes in hematopoiesis in wild-type and HDC-deficient (HDC-KO) mice. NBP treatment did not induce anemia in wild-type or HDC-KO mice, but did produce a g...
Source: International Journal of Hematology - January 4, 2021 Category: Hematology Authors: Otsuka H, Endo Y, Ohtsu H, Inoue S, Noguchi S, Nakamura M, Soeta S Tags: Int J Hematol Source Type: research

Auer rods aid in the diagnosis of mixed phenotype acute leukemia.
PMID: 33398632 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 4, 2021 Category: Hematology Authors: Feng Y, Fu J Tags: Int J Hematol Source Type: research

Dasatinib induces endothelial-to-mesenchymal transition in human vascular-endothelial cells: counteracted by cotreatment with bosutinib.
Abstract Adverse vascular events have become a serious clinical problem in chronic myeloid leukemia (CML) patients who receive certain BCR/ABL1 tyrosine kinase inhibitors (TKIs). Studies have shown that endothelial-to-mesenchymal transition (EndMT) can contribute to various vascular diseases. We investigated the effects of TKIs on the development of EndMT in human vascular-endothelial cells (VECs). Exposure of VECs to dasatinib, but not to other TKIs, produced a significant increase in the formation of spindle-shaped cells. This effect was accompanied by a significant increase in expression of the EndMT inducer tr...
Source: International Journal of Hematology - January 3, 2021 Category: Hematology Authors: Alkebsi L, Wang X, Ohkawara H, Fukatsu M, Mori H, Ikezoe T Tags: Int J Hematol Source Type: research

Effect of donor type on volume of blood transfusions required after allogeneic hematopoietic cell transplantation.
Abstract We reviewed blood product use in 729 consecutive allogeneic hematopoietic cell transplantation (allo-HCT) recipients at our center to assess the volume of red blood cells (RBCs) and platelets required after allo-HCT. The median number of bags required by day 30 was 4 for RBCs (range 0-22) and 9.5 for platelets (0-53). Multivariate analysis showed that related peripheral blood stem cell transplantation (PBSCT) required a significantly lower RBC transfusion volume by day 30 compared to unrelated bone marrow transplantation (UBMT). PBSCT from haplo-identical related donors and cord blood transplantation (CBT...
Source: International Journal of Hematology - January 3, 2021 Category: Hematology Authors: Kurosawa S, Yamaguchi T, Nakabayashi S, Kasane M, Tsubokura M, Iwashita N, Minakawa Y, Ohtake R, Kawamura K, Nishioka Y, Takeda W, Hirakawa T, Aoki J, Ito A, Tanaka T, Inamoto Y, Kim SW, Kojima M, Takanashi M, Fukuda T Tags: Int J Hematol Source Type: research

Safety profile of brentuximab vedotin in Japanese patients with relapsed/refractory Hodgkin lymphoma or systemic anaplastic large cell lymphoma: a post-marketing surveillance study.
This study showed that BV has an acceptable safety profile in Japanese patients with R/R HL and R/R sALCL in the clinical practice setting. However, close monitoring rare, but potentially fatal, ADRs such as pulmonary toxicity may be warranted, especially in patients with prior or ongoing pulmonary disorders. PMID: 33392974 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - January 3, 2021 Category: Hematology Authors: Izutsu K, Ogura M, Tobinai K, Hatake K, Sakamoto S, Nishimura M, Hoshino M Tags: Int J Hematol Source Type: research

Risk factors for delayed elimination of high-dose methotrexate in childhood acute lymphoblastic leukemia and lymphoma.
Abstract High-dose methotrexate (HD-MTX) therapy is widely used in patients with acute lymphoblastic leukemia (ALL) and lymphoma. However, some patients experience delayed MTX elimination, which requires treatment suspension or dose reduction to avoid organ damage. This single-center retrospective analysis reviewed the clinical data of 88 children with ALL or non-Hodgkin lymphoma who received a total of 269 courses of HD-MTX therapy between April 2008 and April 2019. HD-MTX was defined as MTX administration at 2.0, 3.0, or 5.0 g/m2 over a 24-h period, and delayed MTX elimination was defined as a serum MTX con...
Source: International Journal of Hematology - January 3, 2021 Category: Hematology Authors: Nakano T, Kobayashi R, Matsushima S, Hori D, Yanagi M, Suzuki D, Kobayashi K Tags: Int J Hematol Source Type: research

High incidence of venous thrombosis in patients with moderate-to-severe COVID-19.
Abstract Increasing evidence suggests that COVID-19 may be associated with venous thromboembolism, and much data exists regarding high incidence of venous thrombosis in critical COVID-19 patients. However, evidence on this complication in less severe patients is not widely available. The aim of this study was to investigate the incidence of deep-vein thrombosis (DVT) in patients with moderate-to-severe COVID-19, to assess the prevalence of DVT with duplex ultrasound, and to compare patients with DVT and those without it using lung computerized tomography (CT), clinical information and lab data. The subjects of thi...
Source: International Journal of Hematology - January 3, 2021 Category: Hematology Authors: Kerbikov O, Orekhov P, Borskaya E, Nosenko N Tags: Int J Hematol Source Type: research

A study of carfilzomib and dexamethasone in patients with relapsed and refractory multiple myeloma in China.
Abstract The second-generation proteasome inhibitor carfilzomib produces superior outcomes in relapsed or refractory multiple myeloma (MM). We conducted a single-arm trial of twice-weekly carfilzomib (27 mg/m2)-dexamethasone (Kd27) for relapsed and refractory MM in China. Kd27 was administered in 28-day cycles to 123 patients previously treated with ≥ 2 other regimens, including treatment with bortezomib and an immunomodulatory drug, and refractory to their most recent therapy. Overall response rate (ORR) was the primary endpoint; progression-free survival (PFS) and overall survival (OS) were...
Source: International Journal of Hematology - January 3, 2021 Category: Hematology Authors: Du J, Fang B, Li J, Jin J, Wang S, Zou D, Cai Z, Wang H, Hu J, Li W, Fu C, Shao Z, Xia Z, Liu P, Niu T, Tang ET, Kimball AS, Hou J, Chen W Tags: Int J Hematol Source Type: research

A case of JAK2V617F-positive essential thrombocythemia where allele burden was reduced by a PD-1 inhibitor.
Abstract The Janus kinase/signal transducers and activators of transcription signaling pathway induces programmed death ligand-1 (PD-L1) expression. JAK2 mutation at position 617 (JAK2V617) is a frequent driver of myeloproliferative neoplasms (MPN) through PD-L1 expression. Although PD-1 inhibitors should be effective against MPN with JAK2V617F mutation, this has not yet been reported in humans. Thus, we assessed the efficacy of a PD-1 inhibitor in a lung cancer patient with JAK2V617F-positive essential thrombocythemia (ET). A 71-year-old man was diagnosed with ET, and with lung carcinoma 3 years later. After...
Source: International Journal of Hematology - January 3, 2021 Category: Hematology Authors: Hara R, Kawada H, Kikuti YY, Kikkawa E, Harada K, Aoyama Y, Ogiya D, Toyosaki M, Suzuki R, Machida S, Ohmachi K, Onizuka M, Ogawa Y, Masuda R, Iwazaki M, Nakamura N, Ando K Tags: Int J Hematol Source Type: research

Screening for M-proteinemia consisting of monoclonal gammopathy of undetermined significance and multiple myeloma for 30  years among atomic bomb survivors in Hiroshima.
Screening for M-proteinemia consisting of monoclonal gammopathy of undetermined significance and multiple myeloma for 30 years among atomic bomb survivors in Hiroshima. Int J Hematol. 2021 Jan 03;: Authors: Fujimura K, Sugiyama A, Akita T, Ohisa M, Nagashima S, Katayama K, Maeda R, Tanaka J Abstract Monoclonal gammopathy (M-proteinemia) is a premalignant plasma cell disorder. The prevalence of M-proteinemia increases with age and is affected by genetic or environmental factors. Atomic bomb (A-bomb) survivors in Hiroshima are in an age range when they are susceptible to M-proteinemia. The prevalen...
Source: International Journal of Hematology - January 3, 2021 Category: Hematology Authors: Fujimura K, Sugiyama A, Akita T, Ohisa M, Nagashima S, Katayama K, Maeda R, Tanaka J Tags: Int J Hematol Source Type: research

CXCR4 expression by mesenchymal stromal cells is lost after use of enzymatic dissociation agents, but preserved by use of non-enzymatic methods.
ts-Kaya FSF Abstract In recent years, multipotent mesenchymal stromal cells (MSCs) have demonstrated tremendous potential for use in regenerative medicine. CXCR4, the receptor for CXCL12, is highly expressed by bone marrow (BM) MSCs and the CXCR4/CXCL12 axis has been shown to be important for migration and homing of BM-MSCs. Typically, MSCs used for clinical applications are collected after culture expansion using enzymatic methods, such as trypsin. Here, we compared different commercially available enzymatic and non-enzymatic methods for collection and dissociation of MSCs from culture plastics and their effects ...
Source: International Journal of Hematology - January 3, 2021 Category: Hematology Authors: Pervin B, Aydın G, Visser T, Uçkan-Çetinkaya D, Aerts-Kaya FSF Tags: Int J Hematol Source Type: research

Clinical impacts of the mutational spectrum in Japanese patients with primary myelofibrosis.
Abstract Patients with primary myelofibrosis (PMF) have a poorer prognosis than those with other subtypes of myeloproliferative neoplasms (MPNs). To investigate the relationship between gene mutations and the prognosis of Japanese PMF patients, we analyzed mutations in 72 regions located in 14 MPN-relevant genes (CSF3R, MPL, JAK2, CALR, DNMT3A, TET2, EZH2, ASXL1, IDH1/2, SRSF2, SF3B1, U2AF1, and TP53) utilizing a target resequencing platform. In our cohort, ASXL1 mutations were more frequently detected in both overt and prefibrotic PMF patients than other mutations. The frequency of ASXL1 mutations was slightly hi...
Source: International Journal of Hematology - January 2, 2021 Category: Hematology Authors: Morishita S, Ochiai T, Misawa K, Osaga S, Inano T, Fukuda Y, Edahiro Y, Ohsaka A, Araki M, Komatsu N Tags: Int J Hematol Source Type: research

Prevalence of and risk factors for urolithiasis in Croatian patients with hemophilia.
in I Abstract Urolithiasis may be more prevalent in patients with hemophilia (PWH) than in age-matched non-hemophilic males. We conducted a cross-sectional evaluation of 92 adult PWH at University Hospital Center Zagreb. The primary objective was to investigate the frequency of urolithiasis in adult PWH, and the secondary objective was to determine associated risk factors. Urolithiasis was diagnosed by ultrasound and other patient- and hemophilia-related parameters were recorded. The prevalence of urolithiasis was significantly higher among PWH than the reported prevalence in the general Croatian population (10.9...
Source: International Journal of Hematology - January 2, 2021 Category: Hematology Authors: Vodanović M, Lucijanić M, Zupančić Šalek S, Pećin I Tags: Int J Hematol Source Type: research

Recent advances in the research and management of sepsis-associated DIC.
Abstract Disseminated intravascular coagulation (DIC) is a common and life-threatening complication in sepsis. Sepsis-associated DIC is recognized as the systemic activation in coagulation with suppressed fibrinolysis that leads to organ dysfunction in combination with systemic intravascular inflammation. In this process, thrombin contributes a key role in connecting both coagulation and inflammation. Endothelial injury, a result of sepsis, causes DIC due to the effect of multiple activated factors that include neutrophils, platelets, and damage-associated molecular patterns. Recent advances in the understanding o...
Source: International Journal of Hematology - January 2, 2021 Category: Hematology Authors: Iba T, Connors JM, Nagaoka I, Levy JH Tags: Int J Hematol Source Type: research