title: [Acquired hemophilia A. A monocentric retrospective study of 39 patients].
abstract: OBJECTIVE
  Acquired haemophilia A (AHA) is a rare bleeding disorder, due to the presence of an inhibitor directed against factor VIII (FVIII). About 50% of the AHA are idiopathic, while the remaining 50% are related to an underlying disorder or condition (autoimmune diseases, malignancies, postpartum, etc.).
  
  
  METHODS
  We report on a monocentric retrospective cohort of 39 patients with AHA. Data were collected and compared to recent published data.
  
  
  RESULTS
  Thirty-nine patients were admitted for AHA between 1993 et 2011. Mean age at diagnosis was 71.3 years, and we noted a marked male predominance. Although the majority of patients presented a bleeding event at diagnosis (94.9%), the hemorrhagic mortality was low (2.6%). On the contrary, immunosuppressive morbidity and mortality were high in this elderly population. There was a clear correlation between initial FVIII inhibitor titer and complete remission delay. We did not identify prognostic factor for global survival.
  
  
  CONCLUSIONS
  AHA is a rare but potentially fatal disorder. Rapidity of diagnosis and treatment initiation is crucial. Morbidity and mortality, particularly of infectious cause, due to immunosuppressive treatment, should lead to consider other available therapeutical options.
authors.affiliation: Service de médecine interne, Hôtel-Dieu, CHU de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
authors.forename: J
authors.initials: J
authors.lastname: Graveleau
authors.forename: M
authors.initials: M
authors.lastname: Trossaërt
authors.forename: C
authors.initials: C
authors.lastname: Leux
authors.forename: A
authors.initials: A
authors.lastname: Masseau
authors.forename: C
authors.initials: C
authors.lastname: Ternisien
authors.forename: A
authors.initials: A
authors.lastname: Néel
authors.forename: M
authors.initials: M
authors.lastname: Fouassier
authors.forename: C
authors.initials: C
authors.lastname: Agard
authors.forename: M
authors.initials: M
authors.lastname: Sigaud
authors.forename: M
authors.initials: M
authors.lastname: Hamidou
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000453:epidemiology; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D004438:Ecchymosis / Q000453:epidemiology; D017707:Erythrocyte Transfusion / Q000706:statistics & numerical data; D005169:Factor VIII / Q000037:antagonists & inhibitors; D005260:Female; D005602:France / Q000453:epidemiology; D006406:Hematoma / Q000453:epidemiology; D006417:Hematuria / Q000453:epidemiology; D006454:Hemoglobins / Q000032:analysis; D006467:Hemophilia A / Q000453:epidemiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009135:Muscular Diseases / Q000453:epidemiology; D009369:Neoplasms / Q000453:epidemiology; D006472:Oral Hemorrhage / Q000453:epidemiology; D010265:Paraproteinemias / Q000453:epidemiology; D011379:Prognosis; D012189:Retrospective Studies; D012737:Sex Factors; D015996:Survival Rate
chemical_list: D000305:Adrenal Cortex Hormones; D006454:Hemoglobins; D007166:Immunosuppressive Agents; C078147:F8 protein, human; D003520:Cyclophosphamide; D005169:Factor VIII