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

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