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[自身免疫性溶血性贫血发病机制及治疗的研究进展]

[Advances in understanding the pathogenesis and treatment of autoimmune hemolytic anemia].

作者信息

Ueda Yasutaka

机构信息

Department of Hematology and Oncology, Osaka University Graduate School of Medicine.

出版信息

Rinsho Ketsueki. 2022;63(9):1014-1025. doi: 10.11406/rinketsu.63.1014.

Abstract

Autoimmune hemolytic anemia (AIHA) is caused by damaged red blood cells due to auto-antibodies targeting its membrane proteins. The heterogeneous group of diseases is divided into two types depending on the thermal amplitude of autoantibodies: warm and cold AIHA. Cold AIHA includes cold agglutin disease and paroxysmal nocturnal hemoglobinuria. AIHA is also divided into primary and secondary AIHA depending on its etiology. Recent advances in understanding the pathogenesis have revealed that AIHA brings not only anemia but also thromboembolic risk or impaired quality of life (QOL). This review describes its pathogenesis, diagnostic approach, and treatment strategies based on the latest information.

摘要

自身免疫性溶血性贫血(AIHA)是由自身抗体靶向红细胞膜蛋白导致红细胞受损引起的。这一异质性疾病组根据自身抗体的热幅度分为两种类型:温抗体型和冷抗体型自身免疫性溶血性贫血。冷抗体型自身免疫性溶血性贫血包括冷凝集素病和阵发性睡眠性血红蛋白尿症。根据病因,自身免疫性溶血性贫血也可分为原发性和继发性。在理解发病机制方面的最新进展表明,自身免疫性溶血性贫血不仅会导致贫血,还会带来血栓栓塞风险或生活质量(QOL)受损。本综述基于最新信息描述了其发病机制、诊断方法和治疗策略。

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