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获得性血友病 A:2013 年更新。

Acquired haemophilia A: a 2013 update.

机构信息

Pier Mannuccio Mannucci, MD, Scientific Direction, IRCCS Cà Granda Foundation Maggiore Policlinico Hospital, Via Pace 9, 20122 Milan, Italy, Tel.: +39 02 5503 5414, Fax: +39 02 54 100 125, E-mail:

出版信息

Thromb Haemost. 2013 Dec;110(6):1114-20. doi: 10.1160/TH13-05-0363. Epub 2013 Sep 5.

Abstract

Acquired haemophilia A (AHA) is a rare but often severe bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). AHA occurs more frequently in the elderly and in association with several conditions, such as the post-partum period, malignancies, autoimmune diseases or drug exposure; however, approximately 50% of reported cases are apparently idiopathic. Beside the elimination of the underlying disorder, the therapeutic approach to AHA should be directed toward the control of acute bleed and the eradication of FVIII autoantibody production. In this narrative review, we summarise the current knowledge on the epidemiology, diagnosis and clinical features of AHA, focusing in particular on advances in the management of this challenging bleeding disorder.

摘要

获得性血友病 A(AHA)是一种罕见但常导致严重出血的疾病,由针对凝血因子 VIII(FVIII)的自身抗体引起。AHA 更常发生于老年人,并与多种情况相关,如产后、恶性肿瘤、自身免疫性疾病或药物暴露;然而,约 50%的报告病例显然是特发性的。除消除潜在疾病外,AHA 的治疗方法应针对控制急性出血和消除 FVIII 自身抗体的产生。在这篇叙述性综述中,我们总结了 AHA 的流行病学、诊断和临床特征的现有知识,特别关注这一具有挑战性的出血性疾病管理方面的进展。

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