Phase 1/2 study of venetoclax, a BCL-2 inhibitor, in Japanese patients with relapsed or refractory chronic lymphocytic leukemia and small lymphocytic lymphoma.
Abstract Patients with relapsed/refractory (R/R) chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) have limited treatment options. Venetoclax is a potent BCL-2 inhibitor that induces apoptosis in CLL cells. This open-label, phase 1/2 study (NCT02265731) evaluated the safety, pharmacokinetics, and efficacy of venetoclax in Japanese patients with R/R CLL/SLL. Patients enrolled in phase 1 received 400 mg/day venetoclax monotherapy. Patients enrolled in phase 2 received 400 mg/day venetoclax, plus rituximab. Venetoclax was administered with a weekly stepwise ramp-up in doses. In phase 2,...
Source: International Journal of Hematology - October 23, 2020 Category: Hematology Authors: Izutsu K, Yamamoto K, Kato K, Ishikawa T, Fukuhara N, Terui Y, Choi I, Humphrey K, Kim SY, Okubo S, Ogawa N, Nishimura Y, Salem AH, Maruyama D Tags: Int J Hematol Source Type: research

EWSR1 overexpression is a pro-oncogenic event in multiple myeloma.
Abstract Multiple myeloma (MM) is cytogenetically, genetically and molecularly heterogenous even among subclones in one patient, therefore, it is essential to identify both frequent and patient-specific drivers of molecular abnormality. Following previous molecular investigations, we in this study investigated the expression patterns and function of the Ewing sarcoma breakpoint region 1 (EWSR1) gene in MM. The EWSR1 transcriptional level in CD138-positive myeloma cells was higher in 36.4% of monoclonal gammopathy of undetermined significance, in 67.4% of MM patients compared with normal plasma cells, and significa...
Source: International Journal of Hematology - October 23, 2020 Category: Hematology Authors: Nishiyama D, Chinen Y, Isa R, Fujibayashi Y, Kuwahara-Ota S, Yamaguchi J, Takimoto-Shimomura T, Matsumura-Kimoto Y, Tsukamoto T, Shimura Y, Kobayashi T, Horiike S, Taniwaki M, Handa H, Kuroda J Tags: Int J Hematol Source Type: research

Correction to: Programmed death 1 ligand (PD-L1) in solid cancers after allogeneic hematopoietic stem cell transplantation: a retrospective analysis by the Nagasaki Transplant Group.
Abstract In the original publication of the article, the authors would like to alter the color of characters in the Supplemental Table 1 and 2. PMID: 33085031 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - October 21, 2020 Category: Hematology Authors: Kasai S, Itonaga H, Niino D, Miyoshi H, Kato T, Imanishi D, Fujioka M, Furumoto T, Sato S, Sawayama Y, Taguchi J, Imaizumi Y, Hata T, Yoshida S, Moriuchi Y, Ohshima K, Miyazaki Y Tags: Int J Hematol Source Type: research

Characterization of hereditary red blood cell membranopathies using combined targeted next-generation sequencing and osmotic gradient ektacytometry.
Abstract Hereditary red blood cell (RBC) membranopathies are characterized by mutations in genes encoding skeletal proteins that alter the membrane complex structure. Hereditary spherocytosis (HS) is the most common inherited RBC membranopathy leading to hereditary hemolytic anemia with a worldwide distribution and an estimated prevalence, in Europe, of about 1:2000 individuals. The recent availability of targeted next generation sequencing (t-NGS) and its combination with RBC deformability measured with a laser-assisted optical rotational ektacytometer (LoRRca) has demonstrated to be the most powerful contributio...
Source: International Journal of Hematology - October 19, 2020 Category: Hematology Authors: Vives-Corrons JL, Krishnevskaya E, Rodriguez IH, Ancochea A Tags: Int J Hematol Source Type: research

Salt-inducible kinase inhibition sensitizes human acute myeloid leukemia cells to all-trans retinoic acid-induced differentiation.
Abstract Differentiation therapies with all-trans retinoic acid (ATRA) have been successful in treating acute promyelocytic leukemia, a rare subtype of acute myeloid leukemia (AML). However, their efficacy is limited in the case of other AML subtypes. Here, we show that the combination of ATRA with salt-inducible kinase (SIK) inhibition significantly enhances ATRA-mediated AML differentiation. SIK inhibition augmented the ability of ATRA to induce growth inhibition and G1 cell cycle arrest of AML cells. Moreover, combining ATRA and SIK inhibition synergistically activated the Akt signaling pathway but not the MAPK...
Source: International Journal of Hematology - October 19, 2020 Category: Hematology Authors: Zhang XW, Shen X, Long WY, Xiao H, Li FJ, Xing S, Xiong GL, Yu ZY, Cong YW Tags: Int J Hematol Source Type: research

Inhibition of CDK4/6 and autophagy synergistically induces apoptosis in t(8;21) acute myeloid leukemia cells.
Abstract The t(8;21) translocation is the most common cytogenetic abnormality in acute myeloid leukemia (AML). Although t(8;21) AML patients have a relatively favorable prognosis, relapse is a frequent occurrence, underscoring the need to develop novel therapeutic approaches. Here, we showed that t(8;21) AML is characterized by frequent mutation and overexpression of CCND2. Analysis of 19 AML cell lines showed that t(8;21) AML cells had lower IC50 values for the selective CDK4/6 inhibitors palbociclib and abemaciclib than non-t(8;21) AML cells. CDK4/6 inhibitors caused cell cycle arrest at G1 phase and impaired ce...
Source: International Journal of Hematology - October 17, 2020 Category: Hematology Authors: Nakatani K, Matsuo H, Harata Y, Higashitani M, Koyama A, Noura M, Nishinaka-Arai Y, Kamikubo Y, Adachi S Tags: Int J Hematol Source Type: research

Humanized anti CD-20 as an alternative in chronic management of relapsing thrombotic thrombocytopenic microangiopathy resistant to rituximab due to anti chimeric antibody.
Abstract Acquired Immune thrombotic thrombocytopenic purpura (iTTP) is considered among clinical situations that needs not only urgent treatment in acute setting but also long term management to prevent relapses. Important progresses have been made in management of these patients that are definitely associated with reduced mortality and relapse rate. However, there are still noticeable percentage of patients that may relapse despite application of modern treatment strategies including preemptive rituximab infusions. Hereby, we share our experience concerning a frequently relapsing iTTP due to development of anti-r...
Source: International Journal of Hematology - October 16, 2020 Category: Hematology Authors: Ahmadpoor P, Aglae C, Garo F, Cariou S, Renaud S, Reboul P, Moranne O Tags: Int J Hematol Source Type: research

The impact of autoimmune cytopenias on the clinical course and survival of Hodgkin lymphoma.
;nyi Z, Illés Á Abstract The characteristics of autoimmune cytopenias (AICP) associated with Hodgkin lymphoma (HL) are not thoroughly defined. We retrospectively assessed the clinical features of HL-associated AICPs in 563 HL patients diagnosed over a period of 28 years. We identified 8 cases of autoimmune hemolytic anemia (AIHA) and 8 cases of autoimmune thrombocytopenia among 14 patients altogether. Four (26%) AICPs were present at lymphoma diagnosis, while 11 (74%) cytopenias occurred during follow-up after first-line therapy. The overall incidence of HL-associated AICPs was 2.8%. Nine (75%) ...
Source: International Journal of Hematology - October 15, 2020 Category: Hematology Authors: Pinczés LI, Szabó R, Miltényi Z, Illés Á Tags: Int J Hematol Source Type: research

Medical database analysis of japanese multiple myeloma patients with planned stem cell transplantation (MEDALIST) - a focus on healthcare resource utilization and cost.
This study explored the burden associated with stem cell mobilization, with or without cyclophosphamide (CPA), in patients who intended to receive autologous stem cell transplantation (ASCT) for multiple myeloma (MM). A Japanese health care claims database (MDV) was used to analyze the health care resource utilization patterns and medical cost between 2013 and 2016 (pre-plerixafor launch). The patients were further categorized into groups who received granulocyte-colony stimulating factor (G-CSF) alone or G-CSF + CPA group and analyzed in both mobilization and ASCT phases of treatment. Overall, there were mor...
Source: International Journal of Hematology - October 15, 2020 Category: Hematology Authors: Iida S, Ishida T, Horimoto K, Kazama H, Kim H, Crawford B, Teshima T Tags: Int J Hematol Source Type: research

Direct oral anticoagulants in patients with severe inherited thrombophilia: a single-center cohort study.
In conclusion, the results of our cohort study indicate that full-dose dabigatran or apixaban are effective and safe in patients with severe inherited thrombophilia. PMID: 33040276 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - October 11, 2020 Category: Hematology Authors: Zuk J, Papuga-Szela E, Zareba L, Undas A Tags: Int J Hematol Source Type: research

Transcriptional heterogeneity of clonal plasma cells and immune evasion in immunoglobulin light chain amyloidosis.
Abstract Immunoglobulin light chain amyloidosis (AL amyloidosis) is characterized by the presence of B cells producing amyloidogenic immunoglobulin light chains (LCs). The low frequency of aberrant B cells in AL is often masked by a polyclonal B cell background, making it difficult for treatment. We analyzed the single-cell RNA sequencing data from GEO database to compare the plasma cell (PCs) in four individuals with AL amyloidosis, one AL subject after treatment, and six healthy controls. High interindividual variability in AL-derived PCs in their expression pattern of known overexpressed genes in mult...
Source: International Journal of Hematology - October 10, 2020 Category: Hematology Authors: Wang Y, Xu L, Liu Y, Hu Y, Shi Q, Jin L, Yang L, Wang P, Zhang K, Huang X, Ge Q, Lu J Tags: Int J Hematol Source Type: research

Once-weekly vs. twice-weekly carfilzomib dosing in a subgroup of Japanese relapsed and refractory multiple myeloma patients from a randomized phase 3 trial (A.R.R.O.W.) and comparison with ENDEAVOR.
Abstract A.R.R.O.W. evaluated the superiority of once-weekly carfilzomib plus dexamethasone (Kd) 20/70 mg/m2 vs. twice-weekly Kd 20/27 mg/m2 based on progression-free survival (PFS) in relapsed and/or refractory multiple myeloma patients. Forty Japanese patients (once-weekly arm, n = 26; twice-weekly arm, n = 14) were randomized in A.R.R.O.W. In the Japanese subgroup of A.R.R.O.W., median PFS was 14.8 months (95% confidence interval [CI], 7.5-not evaluable [NE]) and 9.7 months (95% CI, 3.8-NE) in the once- and twice-weekly arms, respectively. The overall response rat...
Source: International Journal of Hematology - October 10, 2020 Category: Hematology Authors: Takezako N, Shibayama H, Handa H, Hagiwara S, Ozaki S, Suzuki K, Kosugi H, Ri M, Sugiura I, Choi I, Miyamoto T, Iida S Tags: Int J Hematol Source Type: research

Clinical features of 47 secondary hemophagocytic lymphohistiocytosis patients complicated with capillary leak syndrome.
Abstract The clinical features of patients with secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with capillary leak syndrome (CLS) remain controversial. The data of 259 sHLH patients were retrospectively analyzed. The clinical manifestations, laboratory findings, treatment, and prognosis of the CLS-sHLH group and non-CLS-sHLH group were compared. The levels of fibrinogen, albumin, and serum calcium in the CLS-sHLH group were lower than in the non-CLS-sHLH group, and serum triglycerides in the CLS-sHLH group were higher than in the non-CLS-sHLH group (P  1.3 g/L (P 
Source: International Journal of Hematology - October 9, 2020 Category: Hematology Authors: Man C, Wang M, Yin G, Huang J, Cheng W, Wu X, Liu L, Gao X, Wang J, Tian T, Duan L, Xu J, Qiu H Tags: Int J Hematol Source Type: research

Analysis of determinant factors of liver fibrosis progression in ex-thalassemic patients.
This study analyzes the liver fibrosis status and the determinants of its progression in ex-thalassemic patients. The liver fibrosis status of 108 pediatric transfusion-dependent β-thalassemia major patients was evaluated before and one year after allo-HSCT using transient elastography (TE). All patients achieved normal hematopoiesis. In univariate analyses, not in all, but in patients developing significant post-HSCT iron overload or hepatic graft-versus-host disease (GvHD), as well as recipients of bone marrow stem cells (BMSC), significant TE increment occurred. In multivariable analyses, through a model with large...
Source: International Journal of Hematology - October 8, 2020 Category: Hematology Authors: Rostami T, Monzavi SM, Poustchi H, Khoshdel AR, Behfar M, Hamidieh AA Tags: Int J Hematol Source Type: research

Intensity of immune/clotting assays relate to multiple antiphospholipid antibody positivity in thrombotic primary antiphospholipid syndrome.
Abstract The dual positivity (DP) and triple positivity (TP) concepts bypass the poor comparability of immune/clotting assay for the laboratory classification of antiphospholipid syndrome (APS). To evaluate intensity of immune/clotting assays and DP/TP through different clinical severity groups (CSG) as follows: (1) non-thrombotic asymptomatic carriers of aPL (N-THR), thrombotic primary APS (THR), deceased (D) for recurrent and fatal thrombosis. Activated partial thromboplastin time ratio (aPTTr), dilute Russell viper venom time ratio (DRVVTr), IgG/IgM anticardiolipin (aCL) and anti β-2-glycoprotein-I (a&beta...
Source: International Journal of Hematology - October 8, 2020 Category: Hematology Authors: Ames PRJ, Merashli M, Tommaso B, Iannaccone L, Marottoli V, Ciampa A Tags: Int J Hematol Source Type: research

Retraction Note to: High-dose therapy and autologous stem cell transplantation for relapsed or high-risk diffuse large B-cell lymphoma: a nationwide survey.
Abstract The original article can be found online. PMID: 33030633 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - October 8, 2020 Category: Hematology Authors: Kim SW, Asakura Y, Tajima K, Iwai T, Taji H, Chou T, Morishima Y, Suzumiya J, Sakamaki H, Suzuki R, Fukuda T Tags: Int J Hematol Source Type: research

Chronic myeloid leukemia in solid organ transplant patients: a case series.
Abstract Solid organ transplant (SOT) has transformed the survival and quality of life of patients with end-organ dysfunction. Lifelong need for immunosuppressive medications prolongs life expectancy, but results in altered immune function and is associated with a higher risk of certain malignancies, including chronic myeloid leukemia (CML). In this article, we report on six patients, aged 41-79 years, diagnosed with CML, from 3 to 132 months post-various organ transplants and treated with different tyrosine kinase inhibitors (TKI), including first generation (i.e., imatinib) and second generation (i.e.,...
Source: International Journal of Hematology - October 5, 2020 Category: Hematology Authors: Amitai I, Abulafia AS, Raanani P, Lipton JH Tags: Int J Hematol Source Type: research

The BCRP inhibitor febuxostat enhances the effect of nilotinib by regulation of intracellular concentration.
Abstract Nilotinib is a substrate of the breast cancer resistance protein (BCRP), which is a drug efflux transporter encoded by ABCG2 and regulates the pharmacokinetics of its substrates. We investigated the interaction between nilotinib and BCRP in chronic myeloid leukemia (CML) cells. An imatinib-resistant K562 cell line (K562/IM-R) treated with nilotinib was analyzed for BCRP expression, proliferation, apoptosis, and intracellular nilotinib concentration. K562/IM-R cells cultured with tyrosine kinase inhibitors (TKIs) showed an increased cell count and retained viability, whereas the growth of parental K562 cel...
Source: International Journal of Hematology - October 5, 2020 Category: Hematology Authors: Ito F, Miura M, Fujioka Y, Abumiya M, Kobayashi T, Takahashi S, Yoshioka T, Kameoka Y, Takahashi N Tags: Int J Hematol Source Type: research

Seroprevalence of Toxoplasma gondii among Japanese adults with hematological diseases.
PMID: 33025462 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - October 5, 2020 Category: Hematology Authors: Mori T, Mikita K, Koda Y, Sakurai M, Saburi M, Hasegawa N, Kato J Tags: Int J Hematol Source Type: research

Bone marrow transplantation from a human leukocyte antigen-mismatched unrelated donor in a case with C1q deficiency associated with refractory systemic lupus erythematosus.
We report the case of a 12-year-old female patient with C1q deficiency presenting with intractable SLE who successfully underwent bone marrow transplantation from a human leukocyte antigen (HLA)-mismatched unrelated donor with an immunosuppressive conditioning regimen based on fludarabine, melphalan, and anti-thymocyte globulin. She developed Grade I graft-versus-host disease, but did not have any transplantation-related morbidity. Complete donor chimerism has been maintained for 2 years after transplantation, leading to the restoration of C1q levels and the resolution of SLE symptoms. Normal C1q mRNA expression was o...
Source: International Journal of Hematology - September 29, 2020 Category: Hematology Authors: Matsumura R, Mochizuki S, Maruyama N, Morishita Y, Kawaguchi H, Okada S, Tsumura M, Kaji S, Shimizu J, Shimada A, Kobayashi M Tags: Int J Hematol Source Type: research

Secondary failure of platelet recovery in patients treated with high-dose thiotepa and busulfan followed by autologous stem cell transplantation.
We report here the occurrence of secondary failure of platelet recovery (SFPR) in three out of 24 patients who received high-dose thiotepa and busulfan followed by ASCT. Although there was no obvious abnormality in the primary platelet engraftment as well as the recovery of other blood cells, they developed SFPR with a median time to onset of day 38, and the platelets gradually recovered over several months with steroid therapy. During the same period, there was no development of SFPR among 50 patients who received ASCT with a conditioning regimen of MEAM (ranimustine, etoposide, cytarabine, and melphalan) or high-dose mel...
Source: International Journal of Hematology - September 25, 2020 Category: Hematology Authors: Wada F, Nishikori M, Hishizawa M, Watanabe M, Aiba A, Kitano T, Shimazu Y, Shindo T, Kondo T, Takaori-Kondo A Tags: Int J Hematol Source Type: research

Ruxolitinib for hematopoietic cell transplantation-associated hemophagocytic lymphohistiocytosis.
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a severe complication after allogeneic hematopoietic cell transplantation (HCT) and can cause graft failure or multi-organ failure. Here, we report two children with refractory HCT-associated HLH treated with ruxolitinib. In the first patient, ruxolitinib resolved fever, cytopenia and hyperferritinemia. In another patient, although severe hepatic failure, which developed and worsened before the administration of ruxolitinib, was irreversible, rapid improvement in fever, leukopenia and hyperferritinemia was observed. Of note, multiplex cytokine profiling showed a...
Source: International Journal of Hematology - September 25, 2020 Category: Hematology Authors: Ono R, Ashiarai M, Hirabayashi S, Mizuki K, Hosoya Y, Yoshihara H, Ohtake J, Mori S, Manabe A, Hasegawa D Tags: Int J Hematol Source Type: research

Optimal treatments for TAFRO syndrome: a retrospective surveillance study in Japan.
Abstract TAFRO syndrome is a systemic inflammatory disorder of unknown etiology characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. Mortality in patients with this syndrome is high; however, an optimal treatment strategy has not been established. To explore the strategy, we retrospectively analyzed 81 patients with TAFRO syndrome registered in the Multicenter Collaborative Retrospective Study for Establishing the Concept of TAFRO Syndrome in Japan by December 2019. Sixty-eight patients received corticosteroid therapy as the first-line treatment, and as ...
Source: International Journal of Hematology - September 23, 2020 Category: Hematology Authors: Fujimoto S, Kawabata H, Sakai T, Yanagisawa H, Nishikori M, Nara K, Ohara S, Tsukamoto N, Kurose N, Yamada S, Takai K, Aoki S, Masaki Y Tags: Int J Hematol Source Type: research

Stealth thrombosis of brain and kidney in a girl with Upshaw-Schulman syndrome not receiving prophylactic plasma infusions.
o M PMID: 32965638 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - September 22, 2020 Category: Hematology Authors: Tanabe S, Fujimura Y, Lämmle B, Kimura T, Isonishi A, Sakai K, Matsumoto M Tags: Int J Hematol Source Type: research

Interferon therapy for pregnant patients with essential thrombocythemia in Japan.
We report the outcomes of nine ET patients with ten consecutive high-risk pregnancies. All patients were successfully managed with IFN-α during their pregnancies. All patients also received aspirin and switched to unfractionated heparin around 36 weeks of gestation. As for the seven pregnancies in which IFN-α was started after detection of pregnancy, median platelet counts decreased from 910 to 573 × 109/L after 2 months of IFN-α therapy, and median platelet counts at the time of delivery for all ten pregnancies was 361 × 109/L. All patients gave birth to healt...
Source: International Journal of Hematology - September 22, 2020 Category: Hematology Authors: Edahiro Y, Yasuda H, Gotoh A, Morishita S, Suzuki T, Takeda J, Ando J, Tsutsui M, Itakura A, Komatsu N Tags: Int J Hematol Source Type: research

Retrospective multi-center study of Adolescent and Young Adult (AYA) Multiple Myeloma in Kansai Myeloma Forum registry.
, Kuroda J, Matsumura I, Kanakura Y, Nomura S, Kansai Myeloma Forum Investigators Abstract We retrospectively analyzed the clinical features and outcomes in a real-world cohort of adolescents and the young adult (AYA) patients (age between 16 and 39 years) with symptomatic multiple myeloma (MM) registered with the Kansai Myeloma Forum. 26 patients had been diagnosed as symptomatic MM out of 3284 patients. The prevalence of AYA-MM was 0.8% in this cohort. 81% of the patients was received stem cell transplantation, which may improve outcome. Anemia and hypercalcemia might be prognostic factors, however International...
Source: International Journal of Hematology - September 20, 2020 Category: Hematology Authors: Nakaya A, Kohara T, Shibayama H, Onda Y, Kanda J, Kaneko H, Imada K, Kida T, Kosugi S, Ishikawa J, Yamamura R, Shimazu Y, Tanaka H, Fuchida SI, Shimura Y, Kiyota M, Wada K, Ito T, Uoshima N, Yagi H, Yoshihara S, Ohta K, Shimazaki C, Hino M, Takaori-Kondo Tags: Int J Hematol Source Type: research

Phase 1 dose escalation trial of volasertib in combination with decitabine in patients with acute myeloid leukemia.
Abstract Polo-like kinase 1 (PLK1) regulates mitotic checkpoints and cell division. PLK1 overexpression is reported in numerous cancers, including acute myeloid leukemia (AML), and is associated with poor prognosis. Volasertib is a selective, potent cell-cycle kinase inhibitor that targets PLK to induce mitotic arrest and apoptosis. This phase 1 trial investigated the maximum tolerated dose (MTD), safety, pharmacokinetics, and anti-leukemic activity of volasertib in combination with decitabine in AML patients aged ≥ 65 years. Thirteen patients were treated with escalating volasertib doses (3&...
Source: International Journal of Hematology - September 19, 2020 Category: Hematology Authors: Cortes J, Podoltsev N, Kantarjian H, Borthakur G, Zeidan AM, Stahl M, Taube T, Fagan N, Rajeswari S, Uy GL Tags: Int J Hematol Source Type: research

Relapsed acute monocytic leukemia presenting as histiocytic morphology.
PMID: 32954486 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - September 19, 2020 Category: Hematology Authors: Liu B, Li T Tags: Int J Hematol Source Type: research

Auer-bodies in cellular components other than typical myeloblasts.
PMID: 32951101 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - September 18, 2020 Category: Hematology Authors: Inaba T, Tsukamoto T, Fujita N, Kuroda J Tags: Int J Hematol Source Type: research

Beneficial tyrosine kinase inhibitor therapy in a patient with relapsed BCR-ABL1-like acute lymphoblastic leukemia with CCDC88C-PDGFRB fusion.
Abstract BCR-ABL1-like acute lymphoblastic leukemia (ALL) is a neoplasm of lymphoblasts committed to the B-cell lineage that lack the BCR-ABL1 translocation but show a pattern of gene expression very similar to that seen in ALL with BCR-ABL1 with poor prognosis. A 22-year-old female was diagnosed with common-B-cell-ALL positive for CD10, CD19, CD22, CD79a, CD34, HLA-DR, and TdT in January 2017, and achieved complete remission (CR) with induction therapy, followed by consolidation therapy and maintenance therapy. In March 2020, 6 months after the completion of maintenance therapy, she relapsed. Inotuzumab ozog...
Source: International Journal of Hematology - September 18, 2020 Category: Hematology Authors: Oya S, Morishige S, Ozawa H, Sasaki K, Semba Y, Yamasaki Y, Nakamura T, Aoyama K, Seki R, Mouri F, Osaki K, Miyamoto T, Maeda T, Nagafuji K Tags: Int J Hematol Source Type: research

Long-term outcome in patients with Fanconi anemia who received hematopoietic stem cell transplantation: a retrospective nationwide analysis.
Abstract We retrospectively analyzed nationwide records of 163 Fanconi anemia (FA) patients [aplastic anemia (AA), n = 118; myelodysplastic syndrome (MDS), n = 30; acute leukemia, n = 15] who underwent first allogeneic hematopoietic stem cell transplantation (HSCT) between 1987 and 2015 in Japan. An alternative donor was used in 119 (73%) patients, and 160 (98%) patients received a non-T-cell-depleted graft. With an 8.7-year median follow-up, 5-year overall survival (OS) was 81%. The 5-year OS was significantly higher in AA patients than in MDS and acute leukemia patients ...
Source: International Journal of Hematology - September 18, 2020 Category: Hematology Authors: Yabe M, Morio T, Tabuchi K, Tomizawa D, Hasegawa D, Ishida H, Yoshida N, Koike T, Takahashi Y, Koh K, Okamoto Y, Sano H, Kato K, Kanda Y, Goto H, Takita J, Miyamura T, Noguchi M, Kato K, Hashii Y, Astuta Y, Yabe H Tags: Int J Hematol Source Type: research

Bullous pemphigoid is a common associated disorder with acquired haemophilia A.
This study suggests that BP is a common association with AHA. These patients respond well to bypassing agent and immunosuppression. However, they have a higher recurrence of factor VIII inhibitor and should be monitored closely for relapse during the tapering period of their immunosuppression. PMID: 32949372 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - September 18, 2020 Category: Hematology Authors: Abdul-Halim NA, Ng HJ Tags: Int J Hematol Source Type: research

Clinical and prognostic significance of t(4;14) translocation in multiple myeloma in the era of novel agents.
Abstract Translocation t(4;14) is an independent prognostic factor for adverse outcome in multiple myeloma (MM). However, reports concerning the therapeutic effects of novel drugs on t(4;14) MM are few. We retrospectively investigated the clinical and prognostic significance of symptomatic MM cases with t(4;14) treated with novel therapies. Ninety-three patients (IgG, 56; IgA, 23; BjP, 14) newly diagnosed with MM were included (median age, 71 years; median observation period, 27.8 months). t(4;14) MM was diagnosed in 17 (IgG, 7; IgA, 9; BjP, 1) patients (18%). An association between t(4;14) and the IgA i...
Source: International Journal of Hematology - September 18, 2020 Category: Hematology Authors: Sato S, Kamata W, Okada S, Tamai Y Tags: Int J Hematol Source Type: research

Pembrolizumab plus lenalidomide and dexamethasone in treatment-naive multiple myeloma (KEYNOTE-185): subgroup analysis in Japanese patients.
i K Abstract The global, randomized, open-label KEYNOTE-185 study closed early after an interim analysis showed an unfavorable benefit-risk profile with pembrolizumab plus lenalidomide and low-dose dexamethasone (Rd) versus Rd alone in treatment-naive, transplant-ineligible multiple myeloma. This subgroup analysis reported outcomes in the Japanese population. Patients were randomly assigned (1:1) to pembrolizumab plus Rd or Rd alone, stratified by age and International Staging System. The primary end point was progression-free survival (PFS). Fifty-two Japanese patients were randomly assigned to pembrolizumab plus...
Source: International Journal of Hematology - September 18, 2020 Category: Hematology Authors: Takezako N, Kosugi H, Matsumoto M, Iida S, Ishikawa T, Kondo Y, Ando K, Miki H, Matsumura I, Sunami K, Teshima T, Iwasaki H, Onishi Y, Kizaki M, Izutsu K, Maruyama D, Tobinai K, Ghori R, Farooqui M, Liao J, Marinello P, Matsuda K, Koh Y, Shimamoto T, Suzu Tags: Int J Hematol Source Type: research

Correction to: Menatetrenone facilitates hematopoietic cell generation in a manner that is dependent on human bone marrow mesenchymal stromal/stem cells.
Abstract In the original publication of the article, the Figs. 4 C, F and 5 B, C were published with unexpected appearance of dots. PMID: 32930953 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - September 14, 2020 Category: Hematology Authors: Fujishiro A, Iwasa M, Fujii S, Maekawa T, Andoh A, Tohyama K, Takaori-Kondo A, Miura Y Tags: Int J Hematol Source Type: research

Expert consensus regarding standardization of sample preparation for clotting time assays.
on for clotting time assays, Japanese society for laboratory hematology., Ieko M, Komiyama Y, Yamazaki S, Katagiri H, Shimazu C, Naito S, Matsuda M, Yuki Y, Miyata K, Arai R, Kumano O, Emmi M, Yoshida T, Tanaka H, Okuhara T, Yamashita T, Sekine Y, Homma M, Kawai Y, Tohyama K Abstract Accurate clotting time assay results are vital, as the test is employed to indicate the amount of oral anticoagulant to be prescribed, while it is also used for screening the hemorrhagic and thrombotic diseases. The procedure chosen for preparation of a patient blood sample including centrifugation can contribute to significant differ...
Source: International Journal of Hematology - September 13, 2020 Category: Hematology Authors: Ieko M, Komiyama Y, Yamazaki S, Katagiri H, Shimazu C, Naito S, Matsuda M, Yuki Y, Miyata K, Arai R, Kumano O, Emmi M, Yoshida T, Tanaka H, Okuhara T, Yamashita T, Sekine Y, Homma M, Kawai Y, Tohyama K, working group for standardization of sample preparat Tags: Int J Hematol Source Type: research

Subcutaneous delivery of daratumumab in Japanese patients with relapsed/refractory multiple myeloma.
Abstract Subcutaneous daratumumab (DARA SC; daratumumab co-formulated with recombinant human hyaluronidase PH20) is administered in ~ 5 min and demonstrates safety and efficacy comparable to intravenous daratumumab, with low infusion-related reaction (IRR) rates in global populations. This open-label, multicenter, phase 1 study is the first evaluation of DARA SC in Japanese patients. Eligible patients had relapsed/refractory multiple myeloma (RRMM; ≥ 2 prior lines of therapy including a proteasome inhibitor and immunomodulatory drug). Patients (N = 6) received DARA...
Source: International Journal of Hematology - September 10, 2020 Category: Hematology Authors: Shibayama H, Matsumoto M, Kosugi H, Shibayama K, Yamazaki H, Iida S Tags: Int J Hematol Source Type: research

Advances in the diagnosis and treatment of disseminated intravascular coagulation in haematological malignancies.
Abstract Haematological malignancies, including acute leukaemia and non-Hodgkin lymphoma, are one of the underlying diseases that frequently cause disseminated intravascular coagulation (DIC), an acquired thrombotic disorder. Concomitant DIC is associated with the severity of the underlying disease and poor prognosis. The Japanese Society on Thrombosis and Hemostasis released the new DIC diagnostic criteria in 2017. This criteria include coagulation markers such as soluble fibrin and the thrombin-antithrombin complex to more accurately evaluate the hypercoagulable state in patients. Among several groups of anticoa...
Source: International Journal of Hematology - September 8, 2020 Category: Hematology Authors: Ikezoe T Tags: Int J Hematol Source Type: research

Clinical impact of frailty on treatment outcomes of elderly patients with relapsed and/or refractory multiple myeloma treated with lenalidomide plus dexamethasone.
In conclusion, frailty could predict clinical outcomes of Rd treatment in elderly patients with RRMM who had failed prior bortezomib. In frail patients, lower CCI in addition to less previous treatment exposure and deep response were associated with better survival. PMID: 32889695 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - September 4, 2020 Category: Hematology Authors: Lee HS, Kim K, Lee JJ, Yoon SS, Bang SM, Kim JS, Eom HS, Yoon DH, Lee Y, Shin HJ, Park Y, Jo JC, Lee WS, Do YR, Mun YC, Lee MH, Kim HJ, Kim SH, Kim MK, Lim SN, Park SK, Yi JH, Lee JH, Min CK, Korean Multiple Myeloma Working Party (KMMWP) Tags: Int J Hematol Source Type: research

Outcome of allogeneic hematopoietic stem cell transplantation in adult patients with paroxysmal nocturnal hemoglobinuria.
Abstract The safety and efficacy of allogeneic hematopoietic stem cell transplantation (HSCT) for paroxysmal nocturnal hemoglobinuria (PNH) remain unclear. Therefore, we retrospectively analyzed the outcomes of 42 adult patients with PNH who underwent allogeneic HSCT using the registry database of the Japan Society for Hematopoietic Cell Transplantation. The median patient age was 32.5 years. The number of packed red cell (PRC) transfusions was 
Source: International Journal of Hematology - September 4, 2020 Category: Hematology Authors: Nakamura Y, Takenaka K, Yamazaki H, Onishi Y, Ozawa Y, Ikegame K, Matsuoka KI, Toubai T, Ueda Y, Kanda Y, Ichinohe T, Atsuta Y, Mori T Tags: Int J Hematol Source Type: research

Cutaneous manifestations of B-cell chronic lymphocytic leukemia.
Abstract Chronic lymphocytic leukemia (CLL) is a malignant lymphoproliferative disease characterized by the accumulation of immature monoclonal B lymphocytes in blood cells, bone marrow, spleen and lymph nodes. This is the most common type of leukemia among the Caucasoid race. When CLL skin lesions occur in about 25% of patients, they are extremely diverse. These lesions can be divided into specific, including infiltration of the skin by leukemic cells and the skin form of Richter's syndrome, secondary skin tumors, nonspecific lesions and associated skin diseases.Leukemic infiltration of the skin in patients with ...
Source: International Journal of Hematology - September 4, 2020 Category: Hematology Authors: Morozova EA, Olisova OY, Nikitin EA Tags: Int J Hematol Source Type: research

Complete metabolic remission in an 84-year old patient with relapsed/refractory diffuse large B-cell lymphoma following combination immunotherapy with lenalidomide plus rituximab.
PMID: 32886277 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - September 3, 2020 Category: Hematology Authors: Tomuleasa C, Iluta S, Pasca S, Roman A, Piciu D, Jitaru C, Teodorescu P, Rus I, Bojan A, Dima D, Zdrenghea M, Petrushev B Tags: Int J Hematol Source Type: research

Evaluation of prognosis following early disease progression in peripheral T-cell lymphoma.
In conclusion, the clinical significance of assessing POD24 in PTCL and the poor prognosis in patients with early disease progression were demonstrated. PMID: 32886278 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - September 3, 2020 Category: Hematology Authors: Suzuki Y, Yano T, Suehiro Y, Iwasaki H, Hidaka M, Otsuka M, Sunami K, Ikeda H, Sawamura M, Ito T, Iida H, Nagai H Tags: Int J Hematol Source Type: research

Pharmacokinetics of intravenous busulfan as condition for hematopoietic stem cell transplantation: comparison between combinations with cyclophosphamide and fludarabine.
Abstract Busulfan (Bu) has been used in combination with fludarabine (Flu; BuFlu) or cyclophosphamide (Cy; BuCy) as conditioning for allogeneic hematopoietic stem cell transplantation (HSCT). This multi-institutional prospective study compared pharmacokinetic (PK) parameters of Bu between BuFlu and BuCy. Plasma Bu concentrations were measured by high-performance liquid chromatography at the first dose of the first and fourth days of intravenous Bu administrations (total of 16 doses of 0.8 mg/kg). Thirty-seven patients were evaluable (BuFlu, N = 18; BuCy, N = 19). The median age was...
Source: International Journal of Hematology - September 3, 2020 Category: Hematology Authors: Kikuchi T, Mori T, Ohwada C, Onoda M, Shimizu H, Yokoyama H, Onizuka M, Koda Y, Kato J, Takeda Y, Hino Y, Mishina T, Sakaida E, Shono K, Nagao Y, Yokota A, Matsumoto K, Morita K, Okamoto S, Kanto Study Group for Cell Therapy (KSGCT) Tags: Int J Hematol Source Type: research

High-dose dexamethasone as a replacement for traditional prednisone as the first-line treatment in children with previously untreated primary immune thrombocytopenia: a prospective, randomized single-center study.
Abstract Immune thrombocytopenia (ITP) is one of the most common acquired immune-mediated bleeding disorders found in children. Prednisone is usually considered a first-line therapeutic agent for ITP in children. Yet, prolonged exposure to prednisone has been associated with certain side effects. This prospective randomized study comparatively assessed the efficacy and safety of short-course high-dose dexamethasone (HDD) and standard prednisone (PDN) as a first-line treatment for children with previously untreated primary ITP. Two hundred eleven children were randomized into the HDD (n = 110) and PDN...
Source: International Journal of Hematology - September 2, 2020 Category: Hematology Authors: Ma J, Fu L, Chen Z, Gu H, Ma J, Wu R Tags: Int J Hematol Source Type: research

Bortezomib and cyclophosphamide based chemo-mobilization in multiple myeloma.
Abstract Hematopoietic stem and progenitor cell (HSPC) mobilization regimens in multiple myeloma typically use filgrastim (GCSF) alone or combination of GCSF with plerixafor or high-dose cyclophosphamide. Murine model and human studies have shown HSPC mobilization potential of bortezomib. A total of 37 patients underwent mobilization using bortezomib 1.3 mg/m2 on day 1, 4, 8 and 11, cyclophosphamide 1 g/m2 on day 8 and 9, and GCSF 10 μg/kg from day 10 (B-Cy-GCSF). This regimen was compared with our earlier cohort of patients where cyclophosphamide was given at dose of 1 g/m2 on day 1 and day...
Source: International Journal of Hematology - September 1, 2020 Category: Hematology Authors: Bagal B, Gokarn A, Punatar S, Das S, Bonda A, Nayak L, Chichra A, Kannan S, Mathew LJ, Tembhare P, Patkar N, Poojary M, Ojha S, Subramanian PG, Gujral S, Khattry N Tags: Int J Hematol Source Type: research

Romiplostim is effective for eltrombopag-refractory aplastic anemia: results of a retrospective study.
Abstract Eltrombopag (EPAG) and romiplostim (ROM), thrombopoietin receptor-agonists with demonstrated efficacy against aplastic anemia (AA) in prospective controlled studies, were authorized in Japan for use in adults with aplastic anemia in 2017 and 2019, respectively. So far, no data are available on the potential contribution of switching from ROM to EPAG or vice versa in terms of efficacy or tolerance. Efficacies and tolerance profiles of ten patients, who failed to respond to the maximum dose of EPAG and then switched to ROM, were evaluated. All ten patients received a maximum dose of ROM (20 μg/kg/we...
Source: International Journal of Hematology - September 1, 2020 Category: Hematology Authors: Ise M, Iizuka H, Kamoda Y, Hirao M, Kida M, Usuki K Tags: Int J Hematol Source Type: research

Intrapatient variability in concentration/dose ratio of tacrolimus predicts transplant-associated thrombotic microangiopathy.
Abstract Tacrolimus (TAC) is essential for prophylaxis of acute graft-versus-host disease (aGVHD) after allogeneic hematopoietic cell transplantation (allo-HSCT). We have sometimes observed large fluctuations in TAC concentration. However, links between the variability in the concentration or the concentration/dose (C/D) ratio of TAC and clinical complications remain ambiguous. To clarify relationships between various parameters of TAC and early complications such as pre-engraftment immune reactions/engraftment syndrome, aGVHD, and transplant-associated thrombotic microangiopathy (TA-TMA), a total of 146 patients ...
Source: International Journal of Hematology - September 1, 2020 Category: Hematology Authors: Sagou K, Fukushima N, Ukai S, Goto M, Ozeki K, Kohno A Tags: Int J Hematol Source Type: research

Use of unapproved or off-label drugs in Japan for the treatment of graft-versus-host disease and post-transplant viral infection.
This study demonstrated that numerous off-label and unapproved drugs have been used as key drugs for GVHD and post-transplant viral infection, and the real world date in the transplant registry may serve as an important asset to regulatory purposes. PMID: 32875487 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - August 31, 2020 Category: Hematology Authors: Kuwatsuka Y, Atsuta Y, Hirakawa A, Uchida N, Inamoto Y, Najima Y, Ikegame K, Eto T, Ozawa Y, Ichinohe T, Inoue M, Kimura T, Okamoto S, Miyamura K, Fukuda T Tags: Int J Hematol Source Type: research

18FDG-PET imaging and histopathology in neuroleukemiosis with acute myeloid leukemia.
PMID: 32865707 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)
Source: International Journal of Hematology - August 30, 2020 Category: Hematology Authors: Kiyoki Y, Matsuoka R, Kaneta T, Nishikii H Tags: Int J Hematol Source Type: research