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Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry

Baudo, Francesco; Collins, Peter; Huth-Kuehne, Angela; Levesque, Herve; Marco, Pascual; Nemes, Laszlo; Pellegrini, Fabio; Tengborn, Lilian LU and Knoebl, Paul (2012) In Blood 120(1). p.39-46
Abstract
Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line... (More)
Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%). (Blood. 2012;120(1):39-46) (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Blood
volume
120
issue
1
pages
39 - 46
publisher
American Society of Hematology
external identifiers
  • wos:000307411100011
  • scopus:84863538387
ISSN
1528-0020
DOI
10.1182/blood-2012-02-408930
language
English
LU publication?
yes
id
8d88f6d5-b499-46ea-b948-2100737ab68c (old id 3073148)
date added to LUP
2012-10-05 07:12:45
date last changed
2017-11-12 03:07:45
@article{8d88f6d5-b499-46ea-b948-2100737ab68c,
  abstract     = {Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%). (Blood. 2012;120(1):39-46)},
  author       = {Baudo, Francesco and Collins, Peter and Huth-Kuehne, Angela and Levesque, Herve and Marco, Pascual and Nemes, Laszlo and Pellegrini, Fabio and Tengborn, Lilian and Knoebl, Paul},
  issn         = {1528-0020},
  language     = {eng},
  number       = {1},
  pages        = {39--46},
  publisher    = {American Society of Hematology},
  series       = {Blood},
  title        = {Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry},
  url          = {http://dx.doi.org/10.1182/blood-2012-02-408930},
  volume       = {120},
  year         = {2012},
}