Therapeutic challenges in acquired factor VIII deficiency

Abstract

Management of acquired hemophilia A is challenging and should be undertaken in close collaboration with a hemophilia center with expertise in the field. Treatment involves controlling and preventing bleeds and using immunosuppression to eradicate the inhibitor. Prompt diagnosis is important to allow early hemostatic treatment and to prevent nonessential invasive procedures. First-line hemostatic treatment should be with a bypassing agent. Recombinant activated factor VII and the activated prothrombin complex concentrate anti-inhibitor coagulant complex (Factor Eight Inhibitor Bypassing Activity, or FEIBA) but equally efficacious but both associated with thrombotic events when used in acquired hemophilia. Immunosuppression should be started as soon as a diagnosis has been confirmed. The combination of steroids and cyclophosphamide may induce more patients into remission than steroids alone. Current data do not suggest that rituximab results in better outcomes. Relapse is common (10%-20%) in the first 6 months after immunosuppression is stopped, and patients need to be followed up regularly to allow early diagnosis and treatment of relapse.