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

Tengborn, Lilian LU ; Baudo, F; Huth-Kühne, A; Knoebl, P; Lévesque, H; Paese, M; Pellegrini, F; Nemes, L and Collins, P (2012) In BJOG : an international journal of obstetrics and gynaecology 119(12). p.1529-1537
Abstract
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... (More)
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. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
BJOG : an international journal of obstetrics and gynaecology
volume
119
issue
12
pages
1529 - 1537
publisher
Wiley-Blackwell
external identifiers
  • wos:000309749500013
  • pmid:22901076
  • scopus:84867581661
ISSN
1471-0528
DOI
10.1111/j.1471-0528.2012.03469.x
language
English
LU publication?
yes
id
cf91bbd9-1ab2-48a5-a60e-9ee251cd29ab (old id 3047429)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/22901076?dopt=Abstract
date added to LUP
2012-09-05 19:58:40
date last changed
2017-03-19 04:22:02
@article{cf91bbd9-1ab2-48a5-a60e-9ee251cd29ab,
  abstract     = {Objective:<br/><br>
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. <br/><br>
<br/><br>
Design:<br/><br>
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. <br/><br>
<br/><br>
Main outcome measures:<br/><br>
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. <br/><br>
<br/><br>
Conclusions:<br/><br>
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.},
  author       = {Tengborn, Lilian and Baudo, F and Huth-Kühne, A and Knoebl, P and Lévesque, H and Paese, M and Pellegrini, F and Nemes, L and Collins, P},
  issn         = {1471-0528},
  language     = {eng},
  number       = {12},
  pages        = {1529--1537},
  publisher    = {Wiley-Blackwell},
  series       = {BJOG : an international journal of obstetrics and gynaecology},
  title        = {Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry.},
  url          = {http://dx.doi.org/10.1111/j.1471-0528.2012.03469.x},
  volume       = {119},
  year         = {2012},
}