Advances in the understanding of acquired haemophilia A: implications for clinical practice

Collins, Peter William ORCID: https://orcid.org/0000-0002-6410-1324 and Percy, Charles L. 2010. Advances in the understanding of acquired haemophilia A: implications for clinical practice. British Journal of Haematology 148 (2) , pp. 183-194. 10.1111/j.1365-2141.2009.07915.x

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Abstract

Acquired haemophilia A is an auto-immune disease caused by an inhibitory antibody to factor VIII. Patients with an acquired factor VIII inhibitor are at risk of life- and limb-threatening bleeding until the inhibitor has been eradicated. Management relies on rapid and accurate diagnosis, control of bleeding episodes, investigation for a precipitating cause and eradication of the inhibitor by immunosuppression. Patients should always be managed jointly with a specialist centre even if they present without overt bleeding. Despite an extensive literature, few controlled data are available and management guidelines are predominantly based on case reports, retrospective cohorts and expert opinion. This paper reviews the current literature on incidence, pathogenesis, diagnosis, haemostatic therapy and inhibitor eradication strategies. Potential future developments are discussed.

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Medicine Systems Immunity Research Institute (SIURI)
Subjects:	R Medicine > R Medicine (General)
Uncontrolled Keywords:	acquired haemophilia, factor VIII, inhibitor, bypassing agents, immunosuppression
Publisher:	Wiley-Blackwell
ISSN:	0007-1048
Last Modified:	20 Oct 2022 07:43
URI:	https://orca.cardiff.ac.uk/id/eprint/26186

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