[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

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.295

引用次数: 0

[Extramedullary relapse of acute myeloid leukemia with pericardial effusion after allogeneic hematopoietic stem cell transplantation]. [同种异体造血干细胞移植后急性髓性白血病髓外复发伴心包积液]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.170

Yurie Saitou, Hayato Matsumoto, Takuro Shimbo, Shin Matsuda

引用次数: 0

[Successful treatment with venetoclax and azacitidine in a patient with acute myeloid leukemia with BCR::ABL1 fusion]. 【venetoclax联合阿扎胞苷治疗急性髓系白血病合并BCR::ABL1融合1例成功】。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.184

Kenta Motegi, Akira Katsumi

引用次数: 0

[Clinical features of three cases with primary bone marrow lymphoma]. 【原发性骨髓淋巴瘤3例临床分析】。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.42

Hidetaka Nakagaki, Takahiro Shima, Reiko Yoneda, Masayasu Hayashi, Sae Utsumi, Seiya Hirakawa, Chiaki Kubara, Ken Takigawa, Akihisa Yoshino, Mariko Minami, Yayoi Matsuo, Takuro Kuriyama, Shuichi Taniguchi, Tetsuya Eto

{"title":"[Clinical features of three cases with primary bone marrow lymphoma].","authors":"Hidetaka Nakagaki, Takahiro Shima, Reiko Yoneda, Masayasu Hayashi, Sae Utsumi, Seiya Hirakawa, Chiaki Kubara, Ken Takigawa, Akihisa Yoshino, Mariko Minami, Yayoi Matsuo, Takuro Kuriyama, Shuichi Taniguchi, Tetsuya Eto","doi":"10.11406/rinketsu.66.42","DOIUrl":"10.11406/rinketsu.66.42","url":null,"abstract":"Primary bone marrow lymphoma (PBML) is a malignant lymphoma characterized by proliferation of lymphoma cells exclusively in the bone marrow without lymphadenopathy. Despite the dismal prognosis of PBML, it is a very rare lymphoma with limited evidence concerning its pathophysiology, making accumulation of cases important. We herein report three cases of PBML at our institution. The first patient was an 80-year-old man who presented with hemophagocytic syndrome and pancytopenia at admission, and died of septic shock during initial chemotherapy. The second patient was a 64-year-old man who achieved complete remission with intensive chemotherapies, but relapsed shortly after completing the final chemotherapy course. The third patient was a 66-year-old woman who underwent chemotherapies and allogeneic hematopoietic stem cell transplantation, only to relapse shortly after transplantation. Although early intervention with chemotherapy is essential for PBML treatment, diagnosis of PBML is very challenging due to the absence of lymph node involvement. Moreover, treatment outcomes of existing chemotherapy and transplantation therapies for PBML are still poor. Further accumulation of cases and development of new treatment strategies are desirable.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"42-48"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384276","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

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.71

引用次数: 0