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

Research output: Contribution to journalArticle

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.

Details

Authors
  • P Knoebl
  • M Paese
  • F Baudo
  • P Collins
  • A Huth-Kühne
  • L Nemes
  • F Pellegrini
  • Lilian Tengborn
  • H Lévesque
Organisations
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Cardiac and Cardiovascular Systems

Keywords

  • demographics, treatment, registry, acquired hemophilia, diagnosis, outcome
Original languageEnglish
Pages (from-to)622-631
JournalJournal of Thrombosis and Haemostasis
Volume10
Issue number4
Publication statusPublished - 2012
Publication categoryResearch
Peer-reviewedYes

Bibliographic note

The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Emergency medicine/Medicine/Surgery (013240200)