Management of bleeding in acquired hemophilia A: Results from the European Acquired Haemophilia (EACH2) Registry

Baudo, Francesco, Collins, Peter William ORCID: https://orcid.org/0000-0002-6410-1324, Huth-Kuhne, Angela, Levesque, Herve, Marco, Pascual, Nemes, Laszlo, Pellegrini, Fabio, Tengborn, Lilian and Knoebl, Paul 2012. Management of bleeding in acquired hemophilia A: Results from the European Acquired Haemophilia (EACH2) Registry. Blood 120 (1) , pp. 39-46. 10.1182/blood-2012-02-408930

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Abstract

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%).

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Medicine Systems Immunity Research Institute (SIURI)
Subjects:	R Medicine > RC Internal medicine R Medicine > RM Therapeutics. Pharmacology
Publisher:	American Society of Hematology
ISSN:	0006-4971
Last Modified:	21 Oct 2022 09:42
URI:	https://orca.cardiff.ac.uk/id/eprint/37443

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