[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献

[Clinical characteristics of adolescents and young adults, and the relationship between neutrophil count/ratio and thrombosis in Japanese patients with polycythemia vera and essential thrombocythemia: sub-analyses of JSH-MPN-R18]. [日本真性红细胞增多症和原发性血小板增多症患者的临床特征及中性粒细胞计数/比值与血栓形成的关系：JSH-MPN-R18的亚分析]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.250

Yuka Sugimoto, Keiki Nagaharu

{"title":"[Clinical characteristics of adolescents and young adults, and the relationship between neutrophil count/ratio and thrombosis in Japanese patients with polycythemia vera and essential thrombocythemia: sub-analyses of JSH-MPN-R18].","authors":"Yuka Sugimoto, Keiki Nagaharu","doi":"10.11406/rinketsu.66.250","DOIUrl":"https://doi.org/10.11406/rinketsu.66.250","url":null,"abstract":"This review focuses on the findings of the JSH-MPN-R18 study, a retrospective analysis of 596 patients with polycythemia vera (PV) and 1,152 patients with of essential thrombocythemia (ET). Among the four sub-analyses conducted, one focused on adolescents and young adults (AYA population) with PV and ET, demonstrating that the characteristics of thrombotic events in young Japanese patients differ from those observed in Western populations. Specifically, the proportion of venous thrombosis among all thrombotic events was lower in the Japanese AYA population compared to their Western counterparts. Additionally, an analysis focusing on absolute neutrophil count and neutrophil ratio at diagnosis revealed their utility as predictors of thrombotic events, particularly in low-risk ET patients. These findings should contribute to the development of appropriate management strategies for young PV and ET patients in Japan, as well as to the enhancement of thrombotic risk stratification for PV and ET patients overall.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 4","pages":"250-257"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144048426","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Efficacy and safety of romiplostim in aplastic anemia refractory or intolerant to immunosuppressive therapy with eltrombopag]. [romiplostim治疗再生障碍性贫血的疗效和安全性]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.92

Shuku Sato, Shun Tsunoda, Wataru Kamata, Tomiteru Togano, Yotaro Tamai

引用次数: 0

[Comparison of IPSS-R and IPSS-M in newly diagnosed myelodysplastic neoplasms: a single-center study]. [IPSS-R和IPSS-M在新诊断骨髓增生异常肿瘤中的比较：单中心研究]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.7

Shunsuke Otsuki, Seiichiro Katagiri, Yuya Arai, Shohei Wakamatsu, Mituru Moriyama, Akiko Yamada, Tamiko Suguro, Michiyo Asano, Seiichiro Yoshizawa, Daigo Akahane, Yuko Tanaka, Nahoko Furuya, Hiroaki Fujimoto, Seiichi Okabe, Moritaka Gotoh, Yoshikazu Ito, Hironori Harada, Yuka Harada, Akihiko Gotoh

{"title":"[Comparison of IPSS-R and IPSS-M in newly diagnosed myelodysplastic neoplasms: a single-center study].","authors":"Shunsuke Otsuki, Seiichiro Katagiri, Yuya Arai, Shohei Wakamatsu, Mituru Moriyama, Akiko Yamada, Tamiko Suguro, Michiyo Asano, Seiichiro Yoshizawa, Daigo Akahane, Yuko Tanaka, Nahoko Furuya, Hiroaki Fujimoto, Seiichi Okabe, Moritaka Gotoh, Yoshikazu Ito, Hironori Harada, Yuka Harada, Akihiko Gotoh","doi":"10.11406/rinketsu.66.7","DOIUrl":"10.11406/rinketsu.66.7","url":null,"abstract":"We compared the International Prognostic Scoring System-Revised (IPSS-R) to the International Prognostic Scoring System-Molecular (IPSS-M) in 30 patients with myelodysplastic neoplasms (MDS) newly diagnosed at our institution from January 2021 to February 2023. Molecular analysis was performed by myeloid panel. The median age was 66 years (range: 35-80), and classifications were MDS-LB (n=18), MDS IB-1 (n=1), MDS IB-2 (n=2), MDS-SF3B1 (n=2), MDS-biTP53 (n=1), and MN-pCT (n=6). Each patient had 0 to 8 (median 1) mutations. The most frequently detected mutation was the TET2 mutation, and others detected in>5 patients were U2AF1, TP53, and RUNX1 mutations. IPSS-R classification indicated that 2, 14, 5, 3, and 6 patients were very low, low, intermediate (Int), high, and very high risk, respectively, whereas the IPSS-M classification indicated that 3, 9, 7, 2, 4, and 5 cases were very low, low, moderate-low (ML), moderate-high (MH), high, and very high risk, respectively. Considering IPSS-M ML and MH as the equivalent to IPSS-R Int, 13 (43%) patients had a different risk level in the IPSS-M compared to the IPSS-R. One patient was rated low-risk by IPSS-R, but reclassified as high risk by IPSS-M. It is important to be mindful of this potential for significant discrepancies between risk assessments using IPSS-R and IPSS-M in some cases when making treatment decisions.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"7-11"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143383697","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Identification of genes regulating human CAR-T cell proliferation by genome-wide CRISPR screening]. [通过全基因组CRISPR筛选鉴定人类CAR-T细胞增殖调控基因]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.145

Yoshitaka Adachi

引用次数: 0

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.177

Wakana Takahashi, Nobuaki Fukushima, Tomoya Arakawa, Rintaro Minami, Masaya Numata, Akio Kohno, Kazutaka Ozeki

{"title":"[Unrelated bone marrow transplantation for acute myeloid leukemia evolved from paroxysmal nocturnal hemoglobinuria].","authors":"Wakana Takahashi, Nobuaki Fukushima, Tomoya Arakawa, Rintaro Minami, Masaya Numata, Akio Kohno, Kazutaka Ozeki","doi":"10.11406/rinketsu.66.177","DOIUrl":"10.11406/rinketsu.66.177","url":null,"abstract":"Paroxysmal nocturnal hemoglobinuria (PNH) is a hematopoietic stem cell disease that results from clonal expansion of hematopoietic stem cells with gene mutations. Patients with PNH are known to have an increased risk of developing myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML). We report a case of a 42-year-old woman diagnosed with AML 13 years after a diagnosis of PNH. Gene mutations associated with MDS were detected. She did not achieve complete remission (CR) after induction therapy with idarubicin and cytarabine. Unrelated bone marrow transplantation was performed with a myeloablative conditioning regimen with cyclophosphamide (120 mg/kg) and total body irradiation (12 Gy). Ravulizumab was administered until 47 days after transplantation. She achieved CR after transplantation with complete donor engraftment. The transplantation was successful without severe complications such as graft-versus-host disease or sinusoidal obstruction syndrome. Further accumulation of cases is necessary to determine the efficacy and safety of anti-complement inhibitors in allogeneic hematopoietic stem cell transplantation.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 3","pages":"177-183"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143775019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[A new era in the treatment of aplastic anemia]. 再生障碍性贫血治疗的新时代。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.572

Kohei Hosokawa

{"title":"[A new era in the treatment of aplastic anemia].","authors":"Kohei Hosokawa","doi":"10.11406/rinketsu.66.572","DOIUrl":"10.11406/rinketsu.66.572","url":null,"abstract":"Aplastic anemia (AA) is a rare hematologic disorder characterized by bone marrow hypoplasia and pancytopenia, and is classified into idiopathic and secondary forms. Idiopathic AA is primarily treated with immunosuppressive therapy (IST), thrombopoietin receptor agonists (TPO-RAs), and hematopoietic stem cell transplantation (HSCT). Cyclosporine (CsA) monotherapy is recommended for patients who have mild disease or are moderately transfusion-independent, whereas the combination of anti-thymocyte globulin (ATG) and CsA is the standard treatment for severe disease. In 2023, equine ATG (ATGAM®) was approved in Japan, expanding the options for IST. TPO-RA options for combination therapy with IST now include romiplostim in addition to eltrombopag, and studies have demonstrated the efficacy of triple combination therapy with ATG, CsA, and a TPO-RA has been demonstrated. In the context of HSCT, HLA-haploidentical transplantation using post-transplant cyclophosphamide is increasingly being considered as an option for patients without an HLA-matched donor due to its improved safety and efficacy. This review provides a comprehensive overview of the latest advances in AA treatment, including novel therapeutic strategies, and discusses future therapeutic directions to further improve patient outcomes.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 7","pages":"572-579"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796373","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Molecular mechanism and therapeutic targeting of MLL-rearranged leukemia]. [mll重排白血病的分子机制及治疗靶点]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.657

Akihiko Yokoyama

{"title":"[Molecular mechanism and therapeutic targeting of MLL-rearranged leukemia].","authors":"Akihiko Yokoyama","doi":"10.11406/rinketsu.66.657","DOIUrl":"10.11406/rinketsu.66.657","url":null,"abstract":"MLL gene rearrangements cause malignant leukemia, and continue to pose challenges for both patients and their clinicians. Gene rearrangements result in fusion of MLL with more than 80 different partner genes, whose protein products constitutively activate MLL-target genes such as HOXA9 and MEIS1, thereby transforming hematopoietic progenitors into leukemia cells. Interactome analysis identified MENIN as a common associated factor for both MLL fusions and wild-type MLL. Domain mapping analysis of MLL fusion identified the MENIN binding motif and the CXXC domain as the crucial structures for leukemic transformation. The CXXC domain mediates interaction with unmethylated CpGs, which are clustered in the promoter regions. MLL-MENIN interaction leads to further association with LEDGF, which contains a PWWP domain that binds to di/tri-methylated histone H3 K36 (H3K36me2/3). The PWWP and CXXC domains confer stable binding to CpG-rich promoters containing H3K36me2/3 marks so that MLL fusion proteins are able to recognize their target genes. Thus, the protein complex assembly of MENIN, MLL fusion, and LEDGF is the critical event required for leukemia induction, which provides opportunities for drug-mediated inhibition of the MLL fusion protein complex. Small compounds that interfere with MENIN-MLL interaction have been developed by several pharmaceutical companies. Some are now in clinical trials, and one has even obtained FDA approval.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 7","pages":"657-663"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Successful management of cardiac arrest due to cytokine release syndrome following chimeric antigen receptor T-cell therapy for multiple myeloma]. [嵌合抗原受体t细胞治疗多发性骨髓瘤后细胞因子释放综合征引起的心脏骤停的成功管理]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.749

Yuki Oda, Kota Sato, Kodai Kunisada, Moe Yogo, Tomomi Takei, Mizuki Ogura, Taku Kikuchi, Yu Abe, Nobuhiro Tsukada, Tadao Ishida

{"title":"[Successful management of cardiac arrest due to cytokine release syndrome following chimeric antigen receptor T-cell therapy for multiple myeloma].","authors":"Yuki Oda, Kota Sato, Kodai Kunisada, Moe Yogo, Tomomi Takei, Mizuki Ogura, Taku Kikuchi, Yu Abe, Nobuhiro Tsukada, Tadao Ishida","doi":"10.11406/rinketsu.66.749","DOIUrl":"https://doi.org/10.11406/rinketsu.66.749","url":null,"abstract":"A 58-year-old woman received chimeric antigen receptor T-cell (CAR-T) therapy for triple-class refractory multiple myeloma. Following CAR-T infusion, she developed severe cytokine release syndrome (CRS) and was promptly admitted to the intensive care unit (ICU). Subsequently, she developed immune effector cell-associated neurotoxicity syndrome (ICANS), and then progressed to cardiac arrest. This life-threatening complication was successfully managed with intensive multidisciplinary treatment including mechanical ventilation, vasopressor support, and continuous renal replacement therapy. The patient's high tumor burden at the time of CAR-T infusion likely contributed to the severity of CRS and ICANS. Early intervention with dexamethasone and steroid pulse therapy, along with timely ICU admission, played a pivotal role in the success of treatment. This case highlights the importance of identifying high tumor burden as a risk factor for severe CAR-T-related complications and working closely with medical teams.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 8","pages":"749-755"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144994490","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

[Overview]. (概述)。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.820

Takayuki Ikezoe

引用次数: 0

[Indications for allogeneic stem cell transplantation in Philadelphia chromosome-positive acute lymphoblastic leukemia]. 异基因干细胞移植治疗费城染色体阳性急性淋巴细胞白血病的适应症。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.621

Satoshi Nishiwaki

{"title":"[Indications for allogeneic stem cell transplantation in Philadelphia chromosome-positive acute lymphoblastic leukemia].","authors":"Satoshi Nishiwaki","doi":"10.11406/rinketsu.66.621","DOIUrl":"https://doi.org/10.11406/rinketsu.66.621","url":null,"abstract":"Allogeneic hematopoietic stem cell transplantation (allo-SCT) in first complete remission (CR1) has been the standard treatment for Philadelphia chromosome-positive acute lymphoblastic leukemia. However, the introduction of imatinib, a first-generation tyrosine kinase inhibitor (TKI), dramatically improved outcomes. The subsequent development of next-generation TKIs like dasatinib and ponatinib, as well as the antibody therapy blinatumomab, has further diversified treatment strategies. There is an increasing shift toward avoiding allo-SCT in pediatric patients, both in Japan and internationally. For elderly patients ineligible for allo-SCT, chemotherapy-free regimens combining TKIs and blinatumomab show promise for improving outcomes. Moreover, international studies suggest that young adults may also be able to avoid allo-SCT in CR1. In contrast, current data in Japan are insufficient to support the avoidance of allo-SCT in CR1, and caution is needed when applying findings from overseas. Future efforts should focus on establishing personalized treatment approaches, including risk-stratified transplantation eligibility.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 7","pages":"621-628"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796381","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0