Acquired Hemophilia A: A Permanent Challenge for All Physicians

Abstract

Acquired hemophilia A (AHA) is a rare disease with a prevalence in Europe of 1.5 per million. This diagnosis is significantly delayed in about one-third of all cases, leading to deferred treatment. The main signs of AHA are spontaneous bleeding seen in about two-thirds of all patients. AHA can be lethal in 20% of all symptomatic cases. This patient population’s main standard laboratory finding is a prolonged aPTT (activated prothrombin Time) with otherwise normal coagulation results. In addition, antibodies against FVIII (in Bethesda Units) and a quantitative reduction of FVIII activity are necessary to confirm AHA. The therapy of acute bleeding related to AHA is based on the following main principles: Pharmacologic control of the bleeding is of absolute importance. It can be achieved by administering either recombinant activated FVIIa “bypass therapy”; activated prothrombin complex; or Emicizumab, a bispecific monoclonal antibody. Eradication of the FVIII antibodies should be initiated simultaneously. The combination of steroids with cyclophosphamide leads to the highest eradication rates. Causes of AHA may be related to neoplasms, autoimmune diseases, and pregnancy. We report on a patient who underwent four surgical procedures before the diagnosis of AHA was established.