title: [Harmless bruises? Coagulopathy despite normal INR].
abstract: Acquired hemophilia is a rare but severe bleeding disorder caused by autoantibodies mostly against factor VIII. Clinically it mostly presents with soft tissue and muscular bleeding. Diagnostics include a prolonged aPTT, antibodies against FVIII and a reduced FVIII titre. Acute bleeding is controlled with recombinant factor VIIa (NovoSeven(®)) or activated prothrombin complex (FEIBA(®)), as both bypass FVIII in the coagulation pathway. Treatment to reduce the inhibitor consists of immunosuppression with corticosteroids, cyclophosphamid and can be extended with rituximab.
authors: Departement für Innere Medizin, Klinik für Allgemeine Innere Medizin/Hausarztmedizin, Kantonsspital St. Gallen.
authors: Ladina
authors: L
authors: Greuter
authors: Departement für Innere Medizin, Klinik für Allgemeine Innere Medizin/Hausarztmedizin, Kantonsspital St. Gallen.
authors: Manuela
authors: M
authors: Schöb
authors: Zentrum für Labormedizin, Kantonsspital St. Gallen.
authors: Thomas
authors: T
authors: Lehmann
authors: Departement für Innere Medizin, Klinik für Allgemeine Innere Medizin/Hausarztmedizin, Kantonsspital St. Gallen.
authors: Markus
authors: M
authors: Diethelm
mesh_terms: D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis*; D003288:Contusions / Q000097:blood / Q000209:etiology*; D005260:Female; D006406:Hematoma / Q000097:blood / Q000209:etiology*; D006452:Hemoglobinometry; D006467:Hemophilia A / Q000097:blood / Q000175:diagnosis*; D006801:Humans; D019934:International Normalized Ratio; D008875:Middle Aged; D010314:Partial Thromboplastin Time
keywords: Hemmkörperhämophilie; Immunsuppression; acquired haemophilia; activated prothrombin complex; aktivierter Prothrombinkomplex; immunosuppression; isolated prolonged aPTT; isoliert verlängerte aPTT; recombinant factor VIIa; rekombinanter Faktor VIIa