Autoimmune haemolytic anaemias.

IF 76.9 1区 医学 Q1 MEDICINE, GENERAL & INTERNAL Nature Reviews Disease Primers Pub Date : 2024-11-01 DOI:10.1038/s41572-024-00566-2
Marc Michel, Etienne Crickx, Bruno Fattizzo, Wilma Barcellini
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Abstract

Adult autoimmune haemolytic anaemias (AIHAs) include different subtypes of a rare autoimmune disease in which autoantibodies targeting autoantigens expressed on the membrane of autologous red blood cells (RBCs) are produced, leading to their accelerated destruction. In the presence of haemolytic anaemia, the direct antiglobulin test is the cornerstone of AIHA diagnosis. AIHAs are classified according to the isotype and the thermal optimum of the autoantibody into warm (wAIHAs), cold and mixed AIHAs. wAIHAs, the most frequent type of AIHAs, are associated with underlying conditions in ~50% of cases. In wAIHA, IgG autoantibody reacts with autologous RBCs at 37 °C, leading to antibody-dependent cell-mediated cytotoxicity and increased phagocytosis of RBCs in the spleen. Cold AIHAs include cold agglutinin disease (CAD) and cold agglutinin syndrome (CAS) when there is an underlying condition. CAD and cold agglutinin syndrome are IgM cold antibody-driven AIHAs characterized by classical complement pathway-mediated haemolysis. The management of wAIHAs has long been based around corticosteroids and splenectomy and on symptomatic measures and non-specific cytotoxic agents for CAD. Rituximab and the development of complement inhibitors, such as the anti-C1s antibody sutimlimab, have changed the therapeutic landscape of AIHAs, and new promising targeted therapies are under investigation.

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自身免疫性溶血性贫血。
成人自身免疫性溶血性贫血(AIHA)包括一种罕见自身免疫性疾病的不同亚型,这种疾病会产生针对自体红细胞膜上表达的自身抗原的自身抗体,导致红细胞加速破坏。在出现溶血性贫血时,直接抗球蛋白试验是诊断 AIHA 的基石。AIHA 根据自身抗体的同种型和最适热度分为温性 AIHA(wAIHA)、冷性 AIHA 和混合型 AIHA。wAIHA 是最常见的 AIHA 类型,约 50% 的病例与潜在疾病有关。在 wAIHA 中,IgG 自身抗体在 37 °C 下与自体红细胞发生反应,导致抗体依赖性细胞介导的细胞毒性和脾脏中红细胞吞噬功能增强。冷凝集素血症包括冷凝集素病(CAD)和冷凝集素综合征(CAS)。冷凝集素病和冷凝集素综合征是以经典补体途径介导的溶血为特征的 IgM 冷抗体驱动型 AIHA。长期以来,wAIHAs 的治疗一直以皮质类固醇和脾切除术为基础,而 CAD 则以对症措施和非特异性细胞毒药物为基础。利妥昔单抗(Rituximab)和补体抑制剂(如抗 C1s 抗体 sutimlimab)的开发改变了 AIHAs 的治疗格局,目前正在研究新的有前途的靶向疗法。
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来源期刊
Nature Reviews Disease Primers
Nature Reviews Disease Primers Medicine-General Medicine
CiteScore
76.70
自引率
0.20%
发文量
75
期刊介绍: Nature Reviews Disease Primers, a part of the Nature Reviews journal portfolio, features sections on epidemiology, mechanisms, diagnosis, management, and patient quality of life. The editorial team commissions top researchers — comprising basic scientists and clinical researchers — to write the Primers, which are designed for use by early career researchers, medical students and principal investigators. Each Primer concludes with an Outlook section, highlighting future research directions. Covered medical specialties include Cardiology, Dermatology, Ear, Nose and Throat, Emergency Medicine, Endocrinology, Gastroenterology, Genetic Conditions, Gynaecology and Obstetrics, Hepatology, Haematology, Infectious Diseases, Maxillofacial and Oral Medicine, Nephrology, Neurology, Nutrition, Oncology, Ophthalmology, Orthopaedics, Psychiatry, Respiratory Medicine, Rheumatology, Sleep Medicine, and Urology.
期刊最新文献
Systemic capillary leak syndrome. Systemic capillary leak syndrome. Rett syndrome. Rett syndrome. Autoimmune haemolytic anaemias.
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