Pregnancy‐associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry
- 20 August 2012
- journal article
- research article
- Published by Wiley in BJOG: An International Journal of Obstetrics and Gynaecology
- Vol. 119 (12), 1529-1537
- https://doi.org/10.1111/j.1471-0528.2012.03469.x
Abstract
Please cite this paper as: Tengborn L, Baudo F, Huth‐Kühne A, Knoebl P, Lévesque H, Marco P, Pellegrini F, Nemes L, Collins P on behalf of the EACH2 registry contributors. Pregnancy‐associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry. BJOG 2012;119:1529–1537. 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.Keywords
This publication has 27 references indexed in Scilit:
- Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)Blood, 2012
- Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)Journal of Thrombosis and Haemostasis, 2012
- Consensus recommendations for the diagnosis and treatment of acquired hemophilia ABMC Research Notes, 2010
- International recommendations on the diagnosis and treatment of patients with acquired hemophilia AHaematologica, 2009
- Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' OrganisationBlood, 2006
- The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors OrganisationBritish Journal of Haematology, 2006
- Postpartum acquired factor VIII inhibitors: Results of a surveyAmerican Journal of Hematology, 1998
- Severe intracranial hemorrhage in a newborn infant with transplacental transfer of an acquired factor VIII:C inhibitorThe Journal of Pediatrics, 1995
- DDAVP in acquired hemophilia a: Case report and review of the literatureAmerican Journal of Hematology, 1993
- Normal pregnancy in a patient with a postpartum factor VIII inhibitorAmerican Journal of Hematology, 1987