Abstract
BACKGROUND: Acquired hemophilia is a rare disease caused by the development of auto-antibodies
against factor VIII.
SUBJECTS AND METHODS: We studied the characteristics and outcomes of 34 patients (19
women and 15 men) with acquired hemophilia from 1980 to 1997.
RESULTS: The mean age of the patients was 61 years (range, 22–93 years). An underlying
disease was observed in 18 (53%) patients: 5 patients had cancer, 4 an autoimmune
disorder, 2 a dermatologic disorder, 3 asthma, 3 were postpartum, and 1 had an adverse
reaction to ampicillin. Factor VIII level was <5% in 30 (90%) patients; factor VIII
antibodies were elevated (>10 Bethesda units) in 23 (69%) patients. Bleeding requiring
transfusions was reported in 25 (75%) patients. Human factor VIII was given to 14
patients and porcine factor VIII to 5. Six patients received prothrombin complex concentrates
and one desmopressin. Several immunosuppressive treatments were used, mainly corticosteroids,
cyclophosphamide, and intravenous immunoglobulin. Bleeding stopped in all but one
patient within 2 weeks. Most patients achieved complete remission, although two relapses
were observed subsequently.
CONCLUSION: This large study helps to clarify the presentation and clinical course
of acquired hemophilia. Prospective studies are needed to determine the efficacy of
treatment.
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Article info
Publication history
Published online: August 16, 2004
Accepted:
April 29,
1998
Received in revised form:
April 29,
1998
Received:
December 17,
1997
Identification
Copyright
© 1998 Excerpta Medica Inc. Published by Elsevier Inc. All rights reserved.
