Successful treatment of acquired hemophilia with oral immunosuppressive therapy.
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.
Shaffer
L G
LG
University of Texas-M.D. Anderson Cancer Research Center, Houston, USA.
Phillips
M D
MD
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*
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D005169:Factor VIII; D011241:Prednisone