Acquired hemophilia and its treatment

Abstract

The development of auto-antibodies against clotting factor VIII is a rare disease that occurs predominantly in adults: in pregnant women or people with various immunologic disorders or with no apparent underlying disease. As a consequence of an immune system dysregulation, the evolution of this condition is unpredictable. The aim of treatment is to restore normal factor VIII levels in circulation using desmopressin (DDAVP), massive doses of factor VIII (human or porcine), plasmapheresis and factor VIII infusions. In patients with high-titer inhibitors, products with 'bypasing' activity can be used. The most difficult task is to treat the immune disorder. High-dose infusions of immunoglobulins might be useful, due to the presence of anti-idiotypes to factor VIII inhibitors in the commercial preparations. Corticosteroids and cyclophosphamide are currently the most widely used treatments for the immune disease.