title: Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry.
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%).
authors.affiliation: Thrombosis and Hemostasis Unit, Ospedale Niguarda, Milan, Italy. md9821@mclink.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Baudo
authors.forename: Peter
authors.initials: P
authors.lastname: Collins
authors.forename: Angela
authors.initials: A
authors.lastname: Huth-Kühne
authors.forename: Hervé
authors.initials: H
authors.lastname: Lévesque
authors.forename: Pascual
authors.initials: P
authors.lastname: Marco
authors.forename: László
authors.initials: L
authors.lastname: Nemes
authors.forename: Fabio
authors.initials: F
authors.lastname: Pellegrini
authors.forename: Lilian
authors.initials: L
authors.lastname: Tengborn
authors.forename: Paul
authors.initials: P
authors.lastname: Knoebl
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D005060:Europe / Q000453:epidemiology; D005169:Factor VIII / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy* / Q000401:mortality; D006470:Hemorrhage / Q000188:drug therapy* / Q000401:mortality; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins / Q000627:therapeutic use; D012042:Registries / Q000706:statistics & numerical data*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D001779:Blood Coagulation Factors; D006490:Hemostatics; D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa; D003894:Deamino Arginine Vasopressin