[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献_第7页

[Aberrant mitochondrial dynamics in myeloid neoplasms]. [髓系肿瘤线粒体动力学异常]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.940

Yoshihiro Hayashi

引用次数: 0

[Balancing hemostasis and thrombosis in aging people with hemophilia]. [老年血友病患者止血与血栓的平衡]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.403

Azusa Nagao

{"title":"[Balancing hemostasis and thrombosis in aging people with hemophilia].","authors":"Azusa Nagao","doi":"10.11406/rinketsu.67.403","DOIUrl":"10.11406/rinketsu.67.403","url":null,"abstract":"Advances in hemophilia care have markedly improved bleeding control and extended life expectancy, bringing age-related comorbidities to the forefront. Ischemic heart disease (IHD) and other thrombotic events are no longer rare in real-world practice. While observational studies from Europe and North America report that 5% to 8% of patients experience IHD-related events, earlier Japanese retrospective data suggested a much lower burden. In the nationwide prospective ADVANCE Japan cohort of adults with hemophilia aged ≥40 years, IHD-related events have been observed during follow-up, highlighting the need for contemporary evidence from Japan. Moreover, comparisons between predicted and observed events indicate potential miscalibration of general population-derived risk models (e.g., QRISK and the Suita score) when applied to hemophilia. For acute coronary syndromes and PCI, peri-procedural hemostatic management-ensuring adequate factor VIII/IX activity, optimizing access strategy, and coordinating antithrombotic intensity-is essential. Shortened dual antiplatelet therapy regimens supported by trials in high-bleeding-risk populations may be informative, but extrapolation requires individualized assessment. The role of direct oral anticoagulants, including in patients receiving emicizumab, remains uncertain. Multidisciplinary care and shared decision-making are pivotal to balancing bleeding and thrombosis risks in the aging hemophilia population.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 5","pages":"403-412"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148159346","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

[Pure red cell aplasia in a patient with cold agglutinin disease-associated lymphoproliferative disorder]. [感冒凝集素病相关淋巴细胞增生性疾病患者的纯红细胞发育不全]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.118

Makiko Ogawa, Yuki Osada, Aina Tomori, Taro Mizuki, Sakae Tanosaki, Ken Suzuki

{"title":"[Pure red cell aplasia in a patient with cold agglutinin disease-associated lymphoproliferative disorder].","authors":"Makiko Ogawa, Yuki Osada, Aina Tomori, Taro Mizuki, Sakae Tanosaki, Ken Suzuki","doi":"10.11406/rinketsu.67.118","DOIUrl":"10.11406/rinketsu.67.118","url":null,"abstract":"A 51-year-old woman had been noted to have anemia since 2019 and developed Raynaud's phenomenon during winter or in air-conditioned environments from 2021. In January 2024, she experienced palpitations, tinnitus, dyspnea on exertion, and fatigue. Twenty-three days later, she was urgently admitted to hospital with severe anemia (Hb 2.8 g/dl). She was diagnosed with primary cold agglutinin disease (CAD) and CAD-associated lymphoproliferative disorder (LPD). While she was under observation, undergoing red blood cell transfusion with avoidance of cold exposure, her anemia progressed without worsening of hemolysis and she was diagnosed with pure red cell aplasia (PRCA). Cyclosporine A (CsA) was administered for 10 days without response, and treatment was switched to bendamustine-rituximab (BR) for LPD. Although her LPD improved, her PRCA persisted; CsA was therefore resumed, leading to improvement in the PRCA. In October, her CAD-LPD relapsed with hemolysis, and BR therapy with continued CsA treatment again achieved improvement in the CAD-LPD without worsening of the PRCA. This rare case of primary CAD-associated LPD complicated by PRCA highlights the importance of accurate disease assessment and appropriate treatment strategies.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 2","pages":"118-123"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147358058","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

[Key points in second-line therapy for chronic myeloid leukemia]. 慢性髓系白血病二线治疗要点

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.142

Takaaki Ono

引用次数: 0

[Overview]. (概述)。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.548

Tohru Fujiwara

引用次数: 0

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.517

引用次数: 0

[Diagnosis and management of bleeding disorders caused by hyperfibrinolysis (designated intractable disease No. 347 in Japan)]. 【高纤溶引起的出血性疾病的诊断和治疗（日本指定顽固性疾病No. 347）】。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.606

Yuko Suzuki

{"title":"[Diagnosis and management of bleeding disorders caused by hyperfibrinolysis (designated intractable disease No. 347 in Japan)].","authors":"Yuko Suzuki","doi":"10.11406/rinketsu.67.606","DOIUrl":"https://doi.org/10.11406/rinketsu.67.606","url":null,"abstract":"Hyperfibrinolysis-related bleeding is well recognized in conditions such as the acute phase of trauma, disseminated intravascular coagulation with hyperfibrinolysis, and acute promyelocytic leukemia. In contrast, whether primary hyperfibrinolysis can directly cause bleeding in the absence of an underlying disorder has not been sufficiently investigated, largely due to the lack of an established disease concept and the limited availability of reliable functional assays. Congenital deficiencies of individual fibrinolysis inhibitors-such as plasminogen activator inhibitor-1 (PAI-1) deficiency, α2-plasmin inhibitor (α2-PI) deficiency, and thrombomodulin (TM) disorders leading to impaired activation of thrombin-activatable fibrinolysis inhibitor (TAFI)-are extremely rare but are known to result in severe bleeding manifestations. Based on functional assessment assays for these conditions, we have proposed a novel disease entity, \"bleeding disorders caused by hyperfibrinolysis,\" and established diagnostic criteria. This entity was designated in Japan as an intractable disease (No. 347), effective April 2025. This review outlines the clinical significance and diagnostic framework of this disorder and highlights the potential contribution of fibrinolytic dysfunction to bleeding disorder of unknown cause (BDUC).","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 6","pages":"606-614"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414272","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

[Fibrin-associated large B-cell lymphoma confined to a left atrial myxoma showing durable remission after surgical resection alone]. [纤维蛋白相关大b细胞淋巴瘤局限于左心房黏液瘤，仅手术切除后可持久缓解]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.316

Natsumi Sakai, Masaaki Hotta, Sumire Inoue, Yuya Oshima, Masahiro Yoshimoto, Ayako Iwama, Yukie Tsubokura, Hideaki Yoshimura, Shinya Fujita, Atsushi Satake, Tomoki Ito

{"title":"[Fibrin-associated large B-cell lymphoma confined to a left atrial myxoma showing durable remission after surgical resection alone].","authors":"Natsumi Sakai, Masaaki Hotta, Sumire Inoue, Yuya Oshima, Masahiro Yoshimoto, Ayako Iwama, Yukie Tsubokura, Hideaki Yoshimura, Shinya Fujita, Atsushi Satake, Tomoki Ito","doi":"10.11406/rinketsu.67.316","DOIUrl":"10.11406/rinketsu.67.316","url":null,"abstract":"Fibrin-associated large B-cell lymphoma is an extremely rare subtype of LBCL, characterized by distinctive pathological findings and symptoms, and is generally considered to follow an indolent clinical course. Here we report a case of fibrin-associated large B-cell lymphoma arising in association with an incidentally discovered left atrial myxoma, which has remained in remission with surgical resection alone. The patient was an 84-year-old woman. In September 2023, a routine preoperative chest X-ray performed prior to surgery for osteoarthritis of the shoulder revealed an abnormal shadow. Subsequent echocardiography demonstrated an intracardiac mass in the left atrium, and the patient was referred to our cardiology department. Echocardiography at our hospital revealed a pedunculated mass attached to the atrial septum, consistent with a diagnosis of left atrial myxoma. The mass measured approximately 80 mm and prolapsed between the left atrium and ventricle. Given the risk of obstruction, urgent surgical resection was performed. Histopathological examination of the resected specimen confirmed the diagnosis of fibrin-associated large B-cell lymphoma, and the patient was subsequently referred to the hematology department. A systemic workup revealed no evidence of additional disease. She has remained disease-free without any further therapy.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 4","pages":"316-320"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147936401","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

[Strategies to overcome CAR-T cell exhaustion and terminal differentiation]. [克服CAR-T细胞耗竭和终末分化的策略]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.335

Yuki Kagoya

引用次数: 0

[Intrathecal immunoglobulin administration for refractory viral central nervous system infection after allogeneic hematopoietic stem cell transplantation]. [异体造血干细胞移植后顽固性病毒性中枢神经系统感染的鞘内免疫球蛋白治疗]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.910

Masaaki Matsuo, Shinsuke Kusakabe, Ryumei Kurashige, Kentaro Fukushima, Keiichi Nakata, Akihisa Hino, Hidenori Kasahara, Tomoaki Ueda, Jiro Fujita, Naoki Hosen

引用次数: 0