{"title":"Hémophilie acquise : quoi de neuf en 2024 ?","authors":"Hervé Lévesque , Benoit Guillet , Roseline d’Oiron , Ygal Benhamou","doi":"10.1016/j.revmed.2024.06.005","DOIUrl":null,"url":null,"abstract":"<div><div>Acquired hemophilia A (AHA) is a rare autoimmune disorder due to autoantibodies against factor VIII, with a high mortality risk. It should be suspected in subjects with abnormal bleedings, especially subcutaneous bleed associated with prolonged activated partial thromboplastin time (aPTT). AHA is often idiopathic but is associated with autoimmune diseases, malignancies, pregnancy and postpartum period or drugs. Treatment is based on haemostatic agents as by-passants agents such as factor VIIa and activated prothrombine concentrate complex or recombinant porcine factor VIII for severe bleeding. Eradication of inhibitor should be established as soon as the diagnosis is confirmed with steroid alone often associated with cytotoxic agents or rituximab, depending on FVIII activity and inhibitor titer. The purpose of this review is to summarize the epidemiology, etiopathogenesis, diagnosis, treatment of AHA and discuss current recommendations.</div></div>","PeriodicalId":54458,"journal":{"name":"Revue De Medecine Interne","volume":"45 11","pages":"Pages 710-725"},"PeriodicalIF":0.7000,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Revue De Medecine Interne","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0248866324006660","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0
Abstract
Acquired hemophilia A (AHA) is a rare autoimmune disorder due to autoantibodies against factor VIII, with a high mortality risk. It should be suspected in subjects with abnormal bleedings, especially subcutaneous bleed associated with prolonged activated partial thromboplastin time (aPTT). AHA is often idiopathic but is associated with autoimmune diseases, malignancies, pregnancy and postpartum period or drugs. Treatment is based on haemostatic agents as by-passants agents such as factor VIIa and activated prothrombine concentrate complex or recombinant porcine factor VIII for severe bleeding. Eradication of inhibitor should be established as soon as the diagnosis is confirmed with steroid alone often associated with cytotoxic agents or rituximab, depending on FVIII activity and inhibitor titer. The purpose of this review is to summarize the epidemiology, etiopathogenesis, diagnosis, treatment of AHA and discuss current recommendations.
获得性血友病 A（AHA）是一种罕见的自身免疫性疾病，由针对因子 VIII 的自身抗体引起，具有很高的致死风险。如果患者出现异常出血，尤其是伴有活化部分凝血活酶时间（aPTT）延长的皮下出血，则应怀疑是获得性血友病 A。AHA 通常是特发性的，但也与自身免疫性疾病、恶性肿瘤、妊娠和产后或药物有关。治疗以止血剂为基础，如因子 VIIa 和活化凝血酶原浓缩物复合物等旁通剂或重组猪因子 VIII 用于严重出血。根据 FVIII 的活性和抑制剂滴度，一旦确诊，应立即使用类固醇（通常与细胞毒药物或利妥昔单抗联合使用）根除抑制剂。本综述旨在总结 AHA 的流行病学、发病机制、诊断和治疗，并讨论当前的建议。
期刊介绍：
Official journal of the SNFMI, La revue de medecine interne is indexed in the most prestigious databases. It is the most efficient French language journal available for internal medicine specialists who want to expand their knowledge and skills beyond their own discipline. It is also the main French language international medium for French research works. The journal publishes each month editorials, original articles, review articles, short communications, etc. These articles address the fundamental and innumerable facets of internal medicine, spanning all medical specialties. Manuscripts may be submitted in French or in English.
La revue de medecine interne also includes additional issues publishing the proceedings of the two annual French meetings of internal medicine (June and December), as well as thematic issues.
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