Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A

Abstract

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies inhibiting the function of coagulation factor VIII. It is characterized by spontaneous bleeding in patients with no previous family or personal history of bleeding. Although several large registries have collected clinical data on AHA, limited information is available on the optimal management of AHA because controlled clinical trials are lacking. AHA can easily be diagnosed if the problem (prolonged activated partial thromboplastin time in a bleeding patient) is recognized. After the effects of anticoagulants are excluded, low factor VIII activity and the detection of circulating inhibitors confirms the diagnosis. However, lack of familiarity with this rare condition may delay diagnosis and adequate therapy. Treatment of AHA is based on measures for prompt hemostatic control to stop (and prevent) bleeding, immunosuppression to eradicate the autoantibodies, and supportive care for the adverse effects of that treatment and patients' often complex comorbidities. This article gives a comprehensive overview of the current knowledge about the pathophysiology, diagnosis, and treatment of AHA.

Fig. 1

Diagnostic algorithm for acquired hemophilia A. Modified from Kruse-Jarres et al. [7]. AHA acquired hemophilia A, APTT activated partial thromboplastin time, DTI direct thrombin inhibitor, ELISA anti human FVIII antibody enzyme-linked immunosorbent assay, LMWH low molecular weight heparin, PT prothrombin time, TCT thrombin clotting time, VWF von Willebrand factor

Fig. 2

Prognostic factors of patients with acquired hemophilia A. Data from the GTH AH 01/2010 Study [8, 9]. CI confidence interval, CR complete remission, HR hazard ratio, IgA immunoglobulin A, OS overall survival, WHO World Health Organization performance status