自身免疫性溶血性贫血-动脉网

Autoimmune haemolytic anaemias

Nature 等信源发布 2024-11-01 16:26

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AbstractAdult 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.

摘要成人自身免疫性溶血性贫血（AIHAs）包括一种罕见的自身免疫性疾病的不同亚型，其中产生靶向自体红细胞（RBC）膜上表达的自身抗原的自身抗体，导致其加速破坏。在存在溶血性贫血的情况下，直接抗球蛋白试验是AIHA诊断的基石。

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.

AIHA根据自身抗体的同种型和热最佳状态分为温热（wAIHAs），冷和混合AIHA。wAIHAs是最常见的AIHA类型，在约50%的病例中与潜在疾病有关。在wAIHA中，IgG自身抗体在37℃下与自体红细胞反应，导致抗体依赖性细胞介导的细胞毒性和脾脏中红细胞的吞噬作用增加。

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.

当存在潜在疾病时，感冒AIHAs包括冷凝集素病（CAD）和冷凝集素综合征（CAS）。CAD和冷凝集素综合征是IgM冷抗体驱动的AIHA，其特征在于经典补体途径介导的溶血。wAIHAs的管理长期以来一直基于皮质类固醇和脾切除术，以及针对CAD的症状措施和非特异性细胞毒剂。

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..

利妥昔单抗和补体抑制剂（如抗C1s抗体sutimlimab）的开发改变了AIHAs的治疗前景，正在研究新的有前景的靶向治疗方法。。

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Fig. 1: Pathophysiology of warm adult autoimmune haemolytic anaemias.Fig. 2: Pathophysiology of CAD.Fig. 3: Diagnostic procedure for AIHA.Fig. 4: Treatment algorithm for primary wAIHA and CAD.

图1：温暖的成人自身免疫性溶血性贫血的病理生理学。图2:CAD的病理生理学。图3:AIHA的诊断程序。图4：主要wAIHA和CAD的处理算法。

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Download referencesAuthor informationAuthors and AffiliationsDepartment of Internal Medicine and Clinical Immunology, National Reference Centre for Adult Immune Cytopenias, Henri Mondor University Hospital, Assistance Publique Hôpitaux de Paris, Université Paris-Est Créteil, Créteil, FranceMarc Michel & Etienne CrickxHematology Unit, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Department of Oncology and Hemato-Oncology, University of Milan, Milan, ItalyBruno Fattizzo & Wilma BarcelliniDepartment of Oncology and Hemato-Oncology, University of Milan, Milan, ItalyBruno FattizzoAuthorsMarc MichelView author publicationsYou can also search for this author in.

下载参考文献作者信息作者和所属机构国家成人免疫性血细胞减少症参考中心内科和临床免疫学系，亨利蒙多大学医院，巴黎大学圣克鲁斯分校，巴黎圣克鲁斯大学，克罗泰尔，弗朗西马克·米歇尔和艾蒂安·克里克斯血液科，Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico，米兰大学肿瘤学和血液肿瘤学系，米兰，ItalyBruno Fattizzo＆Wilma Barcellini米兰大学肿瘤学和血液肿瘤学系，米兰，ItalyBruno Fattizzo IzzoAuthorsMarcMichelview作者出版物您也可以在中搜索此作者。

PubMed Google ScholarEtienne CrickxView author publicationsYou can also search for this author in

PubMed Google ScholarEtienne CrickxView作者出版物您也可以在

PubMed Google ScholarBruno FattizzoView author publicationsYou can also search for this author in

PubMed Google ScholarBruno FattizzoView作者出版物您也可以在

PubMed Google ScholarWilma BarcelliniView author publicationsYou can also search for this author in

PubMed Google ScholarWilma BarcelliniView作者出版物您也可以在

PubMed Google ScholarContributionsIntroduction (M.M.); Epidemiology (B.F.); Mechanisms/pathophysiology (E.C.); Diagnosis, screening and prevention (W.B.); Management (M.M.); Quality of life (B.F.); Outlook (M.M.).Corresponding authorCorrespondence to

PubMed谷歌学术贡献生产（M.M.）；流行病学（B.F.）；机制/病理生理学（E.C.）；诊断，筛查和预防（W.B.）；管理（M.M.）；生活质量（B.F.）；展望（M.M.）。对应作者对应

Marc Michel.Ethics declarations

马克·米歇尔。道德宣言

Competing interests

相互竞争的利益

M.M. has received consultancy or advisory board honoraria and speaker’s bureau from Alexion, Amgen, Grifols, Johnson and Johnson Novartis, Sanofi, Sobi and UCB. E.C. has received honoraria (advisory boards, speaker fees) from Novartis, UCB and Sanofi. W.B. has received consultancy or advisory board honoraria for Agios, Alexion, Amgen, Incyte, Novartis, Roche, Sanofi and SOBI; has received lecture fees or congress support from Alexion, Amgen, Novartis and Sanofi; and has received research support from Alexion.

M、 M.曾获得亚历克赛（Alexion）、安进（Amgen）、格里弗斯（Grifols）、强生诺华（Johnson and Johnson Novartis）、赛诺菲（Sanofi）、索比（Sobi）和加州大学伯克利分校（UCB）的顾问或顾问委员会酬金和议长办公室。E、 C.从诺华、加州大学伯克利分校和赛诺菲获得酬金（咨询委员会、演讲费）。W、 B.曾获得安捷斯、亚力兄、安进、英杰、诺华、罗氏、赛诺菲和索比的咨询或顾问委员会酬金；已收到亚历克赛（Alexion）、安进（Amgen）、诺华（Novartis）和赛诺菲（Sanofi）的演讲费或国会支持；并获得了亚力兄的研究支持。

B.F. has received consultancy or advisory board honoraria and speaker’s bureau from Agios, Alexion, Apellis, Janssen, Novartis, Roche, Samsung, Sanofi and Sobi..

B、 F.曾获得安捷斯、亚力兄、阿佩利斯、杨森、诺华、罗氏、三星、赛诺菲和索比的顾问或顾问委员会酬金和发言人局。。

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Nature Reviews Disease Primers thanks J. W. Jacobs, D. L. Hansen, S. S. Datta, B. Mayer and the other, anonymous, reviewer for their contribution to the peer review of this work.

《自然评论》疾病引物感谢J.W.Jacobs，D.L.Hansen，S.S.Datta，B.Mayer和另一位匿名审稿人对这项工作的同行评审做出的贡献。

Additional informationInformed consentThe authors affirm that human research participants provided informed consent for publication of the images in Fig. 2.Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.Related linksNCT03075878: https://clinicaltrials.gov/study/NCT03075878NCT03827603:https://clinicaltrials.gov/study/NCT03827603NCT05757570: https://clinicaltrials.gov/study/NCT05757570NCT05922839: https://clinicaltrials.gov/study/NCT05922839NCT06231368: https://clinicaltrials.gov/study/NCT06231368Rights and permissionsSpringer Nature or its licensor (e.g.

其他信息知情同意作者确认，人类研究参与者为图2中的图像的发布提供了知情同意。出版商的注释Springer Nature对于已发布地图和机构隶属关系中的管辖权主张保持中立。相关链接SNCT03075878：https://clinicaltrials.gov/study/NCT03075878NCT03827603:https://clinicaltrials.gov/study/NCT03827603NCT05757570:（笑声）https://clinicaltrials.gov/study/NCT05757570NCT05922839:（笑声）https://clinicaltrials.gov/study/NCT05922839NCT06231368:（笑声）https://clinicaltrials.gov/study/NCT06231368Rights和许可证原告性质或其许可人（例如。

a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.Reprints and permissionsAbout this articleCite this articleMichel, M., Crickx, E., Fattizzo, B.

协会或其他合作伙伴）根据与作者或其他权利持有人的出版协议对本文拥有专有权；本文接受稿件版本的作者自行存档仅受此类出版协议和适用法律的条款管辖。转载和许可本文引用本文Michel，M.，Crickx，E.，Fattizzo，B。

et al. Autoimmune haemolytic anaemias..

等。自身免疫性溶血性贫血。。

Nat Rev Dis Primers 10, 82 (2024). https://doi.org/10.1038/s41572-024-00566-2Download citationAccepted: 27 September 2024Published: 01 November 2024DOI: https://doi.org/10.1038/s41572-024-00566-2Share this articleAnyone you share the following link with will be able to read this content:Get shareable linkSorry, a shareable link is not currently available for this article.Copy to clipboard.

Nat Rev Dis引物10,82（2024）。https://doi.org/10.1038/s41572-024-00566-2Download引文接受日期：2024年9月27日发布日期：2024年11月1日OI：https://doi.org/10.1038/s41572-024-00566-2Share本文与您共享以下链接的任何人都可以阅读此内容：获取可共享链接对不起，本文目前没有可共享的链接。复制到剪贴板。

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