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Thromb Haemost 2013; 110(06): 1114-1120
DOI: 10.1160/TH13-05-0363
Review Article
Schattauer GmbH

Acquired haemophilia A: A 2013 update

Massimo Franchini
1   Department of Transfusion Medicine and Hematology, Carlo Poma Hospital, Mantova, Italy
,
Pier Mannuccio Mannucci
2   Scientific Direction, IRCCS Cà Granda Foundation Maggiore Hospital, Milan, Italy
› Author Affiliations
Further Information

Publication History

Received: 06 May 2013

Accepted after minor revision: 06 August 2013

Publication Date:
30 November 2017 (online)

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Summary

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.

Keywords

Acquired haemophilia A - FVIII - auto-antibodies - bleeding - inhibitors - therapy
 

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