Acquired haemophilia A: A 2013 update

Massimo Franchini; Pier Mannuccio Mannucci

doi:10.1160/TH13-05-0363

Thrombosis and Haemostasis, Table of Contents

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

Review Article

Schattauer GmbH

Acquired haemophilia A: A 2013 update

Authors

Massimo Franchini

¹Department of Transfusion Medicine and Hematology, Carlo Poma Hospital, Mantova, Italy
Pier Mannuccio Mannucci

²Scientific Direction, IRCCS Cà Granda Foundation Maggiore Hospital, Milan, Italy

Abstract

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

Full Text

References

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