自身免疫性溶血性贫血的诊疗进展

doi:10.3969/j.issn.1671-2587.2023.05.020

临床输血与检验 ›› 2023, Vol. 25 ›› Issue (5): 692-698.DOI: 10.3969/j.issn.1671-2587.2023.05.020

自身免疫性溶血性贫血的诊疗进展

江峰锦¹, 白乐², 余泽波³, 王菡³, 兰炯采⁴, 胡雪³

¹重庆市渝北区人民医院;
²内江市第一人民医院;
³重庆医科大学附属第一医院;
⁴南方医科大学南方医院,重庆 400016

收稿日期:2023-06-30 出版日期:2023-10-20 发布日期:2023-11-20
通讯作者: 胡雪,主要从事中西医结合输血研究,（E-mail）huxue@hospital.cqmu.edu.cn。
作者简介:江峰锦,主要从事医学检验、输血技术研究,（E-mail）3392106273@qq.com。并列第一作者：白乐,主要从事临床输血研究,（E-mail）784435100@qq.com。

Advances in Diagnosis and Treatment of Autoimmune Hemolytic Anemia

JIANG Fengjin, BAI Le, YU Zebo, et al

Chongqing Yubei People's Hospital, Chongqing 400016

Received:2023-06-30 Online:2023-10-20 Published:2023-11-20

摘要/Abstract

摘要： 自身免疫性溶血性贫血（autoimmune hemolytic anemia,AIHA）是由自身红细胞抗体介导的,以红细胞加速破坏为主要表现的溶血性疾病。AIHA的诊断主要依据直接抗人球蛋白试验（direct antiglobulin test,DAT）和溶血相关指标。AIHA的治疗方案由疾病的分型和病因决定,包括输血治疗,对症支持治疗及免疫治疗。在免疫治疗中,温抗体型AIHA一线治疗以糖皮质激素为主,二线治疗药物为利妥昔单抗,三线治疗方案为脾切除和细胞毒性免疫抑制剂;冷抗体型AIHA的一线治疗以利妥昔单抗为主,二线治疗为利妥昔单抗联合苯达莫司汀等方案,三线治疗为补体抑制剂。目前的治疗方案特异性不强,治疗效果欠佳,具有副作用。更多的新药包括福他替尼、Orilanolimab（SYNT 001）、Rozanolixizumab（UCB 7665）、Nipocalimab（M281）、PI3K抑制剂、维奈托克、奥法木单抗、阿仑单抗和达雷木单抗正在进行临床试验。此外,中药治疗可能为AIHA的治疗带来新的潜在突破点。

关键词: 自身免疫性溶血性贫血, 糖皮质激素, 利妥昔单抗, 中药

Abstract: Autoimmune hemolytic anemia (AIHA) is a hemolytic disease caused by its own red blood cell antibodies, which is mainly characterized by accelerated destruction of red blood cells. The diagnosis of AIHA is based on direct antiglobulin test (DAT) and hemolysis related indexes.And the treatment for AIHA based on the type and etiology includes blood transfusion,symptomatic supportive care and immunotherapy. In immunotherapy, glucocorticoids are the main first-line therapy, rituximab the second-line therapy, and splenectomy and cytotoxic immunosuppressants the third-line therapy for warm-antibody type; rituximab is the main first-line therapy, rituximab plus bendamustine the second-line therapy and complement inhibitor the third-line therapy for cold-antibody type. The current treatment regimens are not specific and poor efficacy, and there are side effects. New drugs including foctatinib , orilanolimab ( SYNT 001) , rozanolixizumab (UCB7665), nipocalimab (M281) , PI3K inhibitors, venetoclax, ofamulimab, alemtumab and daratumab are undergoing in clinical trials. In addition, traditional Chinese medicine treatment regimens may bring a new potential breakthrough point for the treatment of AIHA.

Key words: AIHA, Glucocorticoids, Rituximab, Chinese medicine

中图分类号:

R556.6

江峰锦, 白乐, 余泽波, 王菡, 兰炯采, 胡雪. 自身免疫性溶血性贫血的诊疗进展[J]. 临床输血与检验, 2023, 25(5): 692-698.

JIANG Fengjin, BAI Le, YU Zebo, et al. Advances in Diagnosis and Treatment of Autoimmune Hemolytic Anemia[J]. JOURNAL OF CLINICAL TRANSFUSION AND LABORATORY MEDICINE, 2023, 25(5): 692-698.

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自身免疫性溶血性贫血的诊疗进展

Advances in Diagnosis and Treatment of Autoimmune Hemolytic Anemia

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