title: Acquired hemophilia due to factor VIII inhibitors in 34 patients.
abstract: BACKGROUND
  Acquired hemophilia is a rare disease caused by the development of auto-antibodies against factor VIII.
  
  
  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.
  
  
  CONCLUSIONS
  This large study helps to clarify the presentation and clinical course of acquired hemophilia. Prospective studies are needed to determine the efficacy of treatment.
authors.affiliation: Hospital Saint Antoine, Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Bossi
authors.forename: J
authors.initials: J
authors.lastname: Cabane
authors.forename: J
authors.initials: J
authors.lastname: Ninet
authors.forename: R
authors.initials: R
authors.lastname: Dhote
authors.forename: T
authors.initials: T
authors.lastname: Hanslik
authors.forename: O
authors.initials: O
authors.lastname: Chosidow
authors.forename: C
authors.initials: C
authors.lastname: Jouan-Flahault
authors.forename: M H
authors.initials: MH
authors.lastname: Horellou
authors.forename: F
authors.initials: F
authors.lastname: Leynadier
authors.forename: E
authors.initials: E
authors.lastname: Liozon
authors.forename: J
authors.initials: J
authors.lastname: Pouchot
authors.forename: J P
authors.initials: JP
authors.lastname: Robin
authors.forename: F
authors.initials: F
authors.lastname: Sanderson
authors.forename: A
authors.initials: A
authors.lastname: Schaeffer
authors.forename: D
authors.initials: D
authors.lastname: Sicard
authors.forename: F
authors.initials: F
authors.lastname: Staikowsky
authors.forename: B
authors.initials: B
authors.lastname: Wechsler
authors.forename: R
authors.initials: R
authors.lastname: Zittoun
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006467:Hemophilia A / Q000150:complications / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies