Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry

BJOG. 2012 Nov;119(12):1529-37. doi: 10.1111/j.1471-0528.2012.03469.x. Epub 2012 Aug 20.


Objective: The European Acquired Haemophilia registry (EACH2) collected data on the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcome of women with acquired haemophilia A (AHA), a rare and often severe bleeding disorder caused by autoantibodies directed against coagulation factor VIII.

Design: Prospective, multi-centre, large-scale, pan-European registry.

Setting: A total of 117 haemophilia centres in 13 European countries.

Population: Pregnancy-associated AHA.

Methods: Data were reported using a web-based electronic case report form. Diagnosis was based on the presence of a prolonged activated partial thromboplastin time, reduced coagulation Factor VIII level and positive inhibitor assay.

Main outcome measures: Presenting characteristics, time to diagnosis, haemostatic treatment and outcome, immunosuppressive treatment and outcome.

Results: The EACH2 registry (n = 501) documented 42 (8.4%) cases of AHA associated with the peripartum period, a median Factor VIII level at diagnosis of 2.5 (range 0-25) IU/dl and inhibitor titre of 7.8 (range 0.7-348) BU/ml. Antepartum inhibitors were evident in eight women. Time to diagnosis of AHA after delivery was 89 (range 21-120) days. First-line haemostatic treatment was successful in 20/23 (87%) women treated. Bleeding episodes resolved in 17/18 (94%) women treated with a bypassing agent and 29/39 (74%) women achieved complete remission with first-line immunosuppressive treatment. Two babies experienced postnatal bleeding, suggesting transplacental transfer of the antibody. All women were alive at last follow-up.

Conclusions: Although rare, pregnancy-associated AHA may cause severe bleeding-related morbidity. Once diagnosed, women respond well to haemostatic treatment with bypassing agents and immunosuppression. Awareness of peripartum AHA requires improvement to facilitate rapid and appropriate management.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antifibrinolytic Agents / therapeutic use
  • Blood Coagulation Factors / therapeutic use
  • Drug Therapy, Combination
  • Europe / epidemiology
  • Factor VIIa / therapeutic use
  • Female
  • Follow-Up Studies
  • Hemophilia A* / diagnosis
  • Hemophilia A* / drug therapy
  • Hemophilia A* / epidemiology
  • Hemophilia A* / etiology
  • Hemostatics / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Kaplan-Meier Estimate
  • Pregnancy
  • Pregnancy Complications, Hematologic* / diagnosis
  • Pregnancy Complications, Hematologic* / drug therapy
  • Pregnancy Complications, Hematologic* / epidemiology
  • Pregnancy Complications, Hematologic* / etiology
  • Prospective Studies
  • Recombinant Proteins / therapeutic use
  • Registries
  • Treatment Outcome


  • Antifibrinolytic Agents
  • Blood Coagulation Factors
  • Hemostatics
  • Immunosuppressive Agents
  • Recombinant Proteins
  • prothrombin complex concentrates
  • recombinant FVIIa
  • Factor VIIa

Supplementary concepts

  • Factor 8 deficiency, acquired