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Demographic and clinical data in acquired hemophilia a: results from the european acquired haemophilia (each2) registry.

Knoebl, P; Paese, M; Baudo, F; Collins, P; Huth-Kühne, A; Nemes, L; Pellegrini, F; Tengborn, Lilian LU and Lévesque, H (2012) In Journal of Thrombosis and Haemostasis 10(4). p.622-631
Abstract
Background: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. Objectives: The European Acquired Hemophilia registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Results: 501 (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between... (More)
Background: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. Objectives: The European Acquired Hemophilia registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Results: 501 (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003-2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. 57% of the non-pregnancy-related cases were male. 474 bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. 477 patients underwent immunosuppression, and 72.6% achieved complete remission. Conclusions: Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
demographics, treatment, registry, acquired hemophilia, diagnosis, outcome
in
Journal of Thrombosis and Haemostasis
volume
10
issue
4
pages
622 - 631
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000302151200015
  • pmid:22321904
  • scopus:84859181554
ISSN
1538-7933
DOI
10.1111/j.1538-7836.2012.04654.x
language
English
LU publication?
yes
id
54a4fa15-3a9f-4eda-8f50-f0cfb44e4402 (old id 2367083)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/22321904?dopt=Abstract
date added to LUP
2012-03-02 10:45:24
date last changed
2017-11-12 03:07:26
@article{54a4fa15-3a9f-4eda-8f50-f0cfb44e4402,
  abstract     = {Background: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. Objectives: The European Acquired Hemophilia registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Results: 501 (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003-2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. 57% of the non-pregnancy-related cases were male. 474 bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. 477 patients underwent immunosuppression, and 72.6% achieved complete remission. Conclusions: Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA.},
  author       = {Knoebl, P and Paese, M and Baudo, F and Collins, P and Huth-Kühne, A and Nemes, L and Pellegrini, F and Tengborn, Lilian and Lévesque, H},
  issn         = {1538-7933},
  keyword      = {demographics,treatment,registry,acquired hemophilia,diagnosis,outcome},
  language     = {eng},
  number       = {4},
  pages        = {622--631},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {Journal of Thrombosis and Haemostasis},
  title        = {Demographic and clinical data in acquired hemophilia a: results from the european acquired haemophilia (each2) registry.},
  url          = {http://dx.doi.org/10.1111/j.1538-7836.2012.04654.x},
  volume       = {10},
  year         = {2012},
}