title: Successful treatment of acquired hemophilia with oral immunosuppressive therapy.
abstract: BACKGROUND
  Acquired autoantibodies to factor VIII in patients without hemophilia are rare, but they cause severe illness and death if not eradicated.
  
  
  OBJECTIVE
  To examine daily therapy with oral cyclophosphamide and prednisone for acquired hemophilia.
  
  
  METHODS
  Case series.
  
  
  METHODS
  Academic medical center.
  
  
  METHODS
  Nine consecutive patients without hemophilia who had severe hemorrhage caused by high titers of factor VIII inhibitors.
  
  
  METHODS
  Daily oral cyclophosphamide and prednisone. Coagulation factors were used only for bleeding.
  
  
  METHODS
  Plasma titers of factor VIII inhibitor, factor VIII activity, and clinical evidence of bleeding.
  
  
  RESULTS
  All patients achieved complete remission, which was defined as loss of residual inhibitor activity and return to a normal titer of factor VIII. Therapy lasted a median of 12 weeks (range, 3 to 37 weeks). Bleeding resolved in a median of 3 weeks (range, 2 to 10 weeks). Median follow-up after discontinuation of therapy was 91 weeks (range, 61 to 164 weeks).
  
  
  CONCLUSIONS
  Daily administration of oral cyclophosphamide and prednisone without empirical factor VIII therapy seems to be an effective and well-tolerated treatment for acquired hemophilia.
authors.affiliation: University of Texas-M.D. Anderson Cancer Research Center, Houston, USA.
authors.forename: L G
authors.initials: LG
authors.lastname: Shaffer
authors.forename: M D
authors.initials: MD
authors.lastname: Phillips
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005169:Factor VIII / Q000276:immunology; D005260:Female; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D006470:Hemorrhage / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use*
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D005169:Factor VIII; D011241:Prednisone