Harmless bruises? Coagulopathy despite normal INR

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.