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Changes in bleeding patterns in von Willebrand disease after institution of long-term replacement therapy: results from the von Willebrand Disease Prophylaxis Network.

Holm, Elena LU ; Abshire, Thomas C; Bowen, Joel; Álvarez, M A Teresa; Bolton-Maggs, Paula; Carcao, Manuel; Federici, Augusto B; Gill, Joan Cox; Halimeh, Susan and Kempton, Christine, et al. (2015) In Blood Coagulation and Fibrinolysis 26(4). p.383-388
Abstract
Clinically, the leading symptom in von Willebrand disease (VWD) is bleeding, chiefly of mucosal type, for example, epistaxis, gingival, or gastrointestinal bleeding, and menorrhagia. In severe forms of VWD with secondary deficiency of factor VIII, spontaneous joint bleeding, resembling that observed in severe haemophilia A, may also be observed. The bleeding patterns of VWD can affect quality of life, and may be life-threatening. The von Willebrand Disease Prophylaxis Network is an international study group formed with the goal of investigating the role of prophylaxis in clinically severe VWD. The objective of the present study is to investigate the response to prophylaxis focusing primarily on epistaxis, joint bleeding, gastrointestinal... (More)
Clinically, the leading symptom in von Willebrand disease (VWD) is bleeding, chiefly of mucosal type, for example, epistaxis, gingival, or gastrointestinal bleeding, and menorrhagia. In severe forms of VWD with secondary deficiency of factor VIII, spontaneous joint bleeding, resembling that observed in severe haemophilia A, may also be observed. The bleeding patterns of VWD can affect quality of life, and may be life-threatening. The von Willebrand Disease Prophylaxis Network is an international study group formed with the goal of investigating the role of prophylaxis in clinically severe VWD. The objective of the present study is to investigate the response to prophylaxis focusing primarily on epistaxis, joint bleeding, gastrointestinal bleeding, and heavy bleeding associated with menses. Data from 105 subjects, 10 enrolled in a prospective study and 95 in a retrospective study between 2008 and 2013, were available for analysis. The median annualized rate reductions in bleeding were significant for epistaxis (P < 0.0001), gastrointestinal bleeding (P = 0.0003), joint bleeding (P < 0.0001), and menorrhagia (P = 0.008). Doses on a group level were approximately the same prior to and during prophylaxis, but more patients with gastrointestinal bleeding had prophylaxis three or more times per week as well as higher dosages. Our study, which primarily used retrospective data, indicates that prospective studies are needed to better delineate the doses and dose intervals that should be used for prophylactic treatment of VWD. (Less)
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published
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Blood Coagulation and Fibrinolysis
volume
26
issue
4
pages
383 - 388
publisher
Lippincott Williams and Wilkins
external identifiers
  • pmid:25688461
  • wos:000353828800005
  • scopus:84929471799
ISSN
1473-5733
DOI
10.1097/MBC.0000000000000257
language
English
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yes
id
209ee6e2-c7d9-4bcc-b90c-f4a094a43c1a (old id 5143485)
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http://www.ncbi.nlm.nih.gov/pubmed/25688461?dopt=Abstract
date added to LUP
2015-03-08 17:38:38
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2017-10-22 03:24:52
@article{209ee6e2-c7d9-4bcc-b90c-f4a094a43c1a,
  abstract     = {Clinically, the leading symptom in von Willebrand disease (VWD) is bleeding, chiefly of mucosal type, for example, epistaxis, gingival, or gastrointestinal bleeding, and menorrhagia. In severe forms of VWD with secondary deficiency of factor VIII, spontaneous joint bleeding, resembling that observed in severe haemophilia A, may also be observed. The bleeding patterns of VWD can affect quality of life, and may be life-threatening. The von Willebrand Disease Prophylaxis Network is an international study group formed with the goal of investigating the role of prophylaxis in clinically severe VWD. The objective of the present study is to investigate the response to prophylaxis focusing primarily on epistaxis, joint bleeding, gastrointestinal bleeding, and heavy bleeding associated with menses. Data from 105 subjects, 10 enrolled in a prospective study and 95 in a retrospective study between 2008 and 2013, were available for analysis. The median annualized rate reductions in bleeding were significant for epistaxis (P &lt; 0.0001), gastrointestinal bleeding (P = 0.0003), joint bleeding (P &lt; 0.0001), and menorrhagia (P = 0.008). Doses on a group level were approximately the same prior to and during prophylaxis, but more patients with gastrointestinal bleeding had prophylaxis three or more times per week as well as higher dosages. Our study, which primarily used retrospective data, indicates that prospective studies are needed to better delineate the doses and dose intervals that should be used for prophylactic treatment of VWD.},
  author       = {Holm, Elena and Abshire, Thomas C and Bowen, Joel and Álvarez, M A Teresa and Bolton-Maggs, Paula and Carcao, Manuel and Federici, Augusto B and Gill, Joan Cox and Halimeh, Susan and Kempton, Christine and Key, Nigel S and Kouides, Peter and Lail, Alice and Landorph, Andrea and Leebeek, Frank and Makris, Michael and Mannucci, Pier and Mauser-Bunschoten, Eveline P and Nugent, Diane and Valentino, Leonard A and Winikoff, Rochelle and Berntorp, Erik},
  issn         = {1473-5733},
  language     = {eng},
  number       = {4},
  pages        = {383--388},
  publisher    = {Lippincott Williams and Wilkins},
  series       = {Blood Coagulation and Fibrinolysis},
  title        = {Changes in bleeding patterns in von Willebrand disease after institution of long-term replacement therapy: results from the von Willebrand Disease Prophylaxis Network.},
  url          = {http://dx.doi.org/10.1097/MBC.0000000000000257},
  volume       = {26},
  year         = {2015},
}