[Treatment strategies for autoimmune hemolytic anemia].

Takayuki Ikezoe
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Abstract

Autoimmune hemolytic anemia (AIHA), which is caused by autoantibodies for red blood cell membrane antigens, is categorized into two forms: warm AIHA, which involves warm antibodies, and cold agglutinin disease (CAD), which involves hemolysis and red blood cell agglutination due to cold agglutinins. The first-line therapy for wAIHA is corticosteroids. Clinical guidelines by the British Society for Haematology recommend rituximab as second-line therapy, but Japanese national health insurance does not cover rituximab for wAIHA. Several new drugs with different mechanisms of action are in clinical development for refractory cases. Some of these drugs inhibit antibody production or promote antibody clearance, while others inhibit erythrophagocytosis. In CAD, anti-complement drugs targeting C1s improve anemia but do not treat peripheral circulatory failure due to erythrocyte aggregation. B-cell-targeted therapies should be used for patients with severe symptoms of these conditions.

自身免疫性溶血性贫血的治疗策略
自身免疫性溶血性贫血(AIHA)是由红细胞膜抗原的自身抗体引起的,分为两种形式:温热抗体引起的自身免疫性溶血性贫血(AIHA)和冷凝集素病(CAD),由于冷凝集素引起的溶血和红细胞凝集。wAIHA的一线治疗是皮质类固醇。英国血液病学会的临床指南推荐利妥昔单抗作为二线治疗,但日本国民健康保险不包括利妥昔单抗治疗wAIHA。几种具有不同作用机制的新药正在临床开发中,用于治疗难治性病例。其中一些药物抑制抗体产生或促进抗体清除,而另一些药物抑制红细胞吞噬。在冠心病中,靶向C1s的抗补体药物可改善贫血,但不能治疗红细胞聚集引起的外周循环衰竭。对于这些症状严重的患者,应采用b细胞靶向治疗。
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