自身免疫性溶血性贫血的治疗  

The treatment strategies of autoimmune hemolytic anemia

作　　者：岳文如 吴婷[1] 王小钦[1] Yue Wenru;Wu Ting;Wang Xiaoqin(Huashan Hospital Affiliated to Fudan University,Shanghai 200040,China)

机构地区：[1]复旦大学附属华山医院,上海200040

出　　处：《中华血液学杂志》2024年第6期615-620,共6页Chinese Journal of Hematology

基　　金：上海市卫生健康委员会科研项目(20234Y0004)。

摘　　要：自身免疫性溶血性贫血(AIHA)是自身抗体和(或)补体吸附于红细胞表面,通过抗原抗体反应加速红细胞破坏而引起的一种溶血性贫血,根据自身抗体类型及抗原抗体反应的最佳温度分为温抗体型、冷抗体型及混合型。糖皮质激素±利妥昔单抗是温抗体型AIHA(wAIHA)的一线治疗方法,脾切除术曾是复发或难治wAIHA的首选二线治疗。但由于各种并发症的发生,利妥昔单抗逐渐成为糖皮质激素治疗失败患者的首选。其他可选治疗包括免疫抑制剂、血浆置换等。糖皮质激素和脾切除术不适用于冷抗体型AIHA,利妥昔单抗单药或联合苯达莫司汀常作为其一线治疗。补体抑制剂苏替利单抗(Sutimlimab)目前已被批准用于治疗冷凝集素病(CAD)。近年来,许多新兴的治疗方案如针对B细胞、浆细胞药物,补体系统抑制剂和抑制吞噬作用的药物等,在有效性和安全性方面展现出巨大潜力,为AIHA的临床治疗提供了新的视角。Autoimmune hemolytic anemia(AIHA)is characterized by the accelerated destruction of erythrocytes due to the presence of antibodies and/or complement that bind to antigens on erythrocytes.It can be subdivided into warm,cold or mixed AIHA based on the type of autoantibody and the optimal temperature of antigen-antibody reaction.Glucocorticoid with or without rituximab is the first-line treatment of warm AIHA(wAIHA),and splenectomy was once the preferred second-line treatment for relapsed or refractory wAIHA.However,due to the various complications of splenectomy,rituximab has gradually become the preferred treatment for patients who have failed glucocorticoid therapy.Other available treatments including immunosuppressants and plasma exchange can be chosen.Rituximab with or without bendamustine is generally taken as the first-line regimen for cold autoimmune hemolytic anemia(cAIHA),while glucocorticoid and splenectomy are ineffective.Sutimlimab,a kind of complement inhibitor,has been approved for the treatment of cold agglutinin disease(CAD).In recent years,many new drugs have emerged as treatment options for AIHA.Emerging therapies,including B-cell-directed therapies,plasma cell-directed therapies,complement inhibitors,and phagocytosis inhibition,provide a new perspective for AIHA therapy,showing great potential for clinical applications.

关 键 词：脾切除术 利妥昔单抗 自身免疫性溶血性贫血 红细胞破坏 血浆置换 抗原抗体反应 糖皮质激素 AIHA 

分 类 号：R556.62[医药卫生—血液循环系统疾病]