Acquired hemophilia and its treatment.
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.
Sultan
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Laboratoire d'Hématologie, CHU La Milétrie, Poitiers, France.
D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000188:drug therapy*; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D005169:Factor VIII / Q000008:administration & dosage / Q000276:immunology* / Q000627:therapeutic use; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy* / Q000276:immunology*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D011247:Pregnancy
D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin