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Prophylaxis and treatment of bleeding complications in von Willebrand disease type 3.

Berntorp, Erik LU (2006) In Seminars in Thrombosis and Hemostasis 32(6). p.621-625
Abstract
Treatment of type 3 von Willebrand disease (vWD) relies on infusion with plasma-derived factor concentrates containing von Willebrand factor (vWF). Patients with types 1 and 2 vWD who do not respond satisfactorily after receiving desmopressin need treatment with concentrates. The rationale for long-term prophylaxis in vWD is obvious: prophylaxis has been successfully used in hemophilia, and joint hemorrhages with development of hemophilic arthropathy can occur, especially in type 3 vWD. In Sweden, prophylaxis for vWD began during the 1960s, and we now have experience from a cohort of 37 patients treated for a median of 11 years (range, 2 to 45 years). The majority of subjects (n = 28) have type 3 vWD. The mean dose used for treatment is 24... (More)
Treatment of type 3 von Willebrand disease (vWD) relies on infusion with plasma-derived factor concentrates containing von Willebrand factor (vWF). Patients with types 1 and 2 vWD who do not respond satisfactorily after receiving desmopressin need treatment with concentrates. The rationale for long-term prophylaxis in vWD is obvious: prophylaxis has been successfully used in hemophilia, and joint hemorrhages with development of hemophilic arthropathy can occur, especially in type 3 vWD. In Sweden, prophylaxis for vWD began during the 1960s, and we now have experience from a cohort of 37 patients treated for a median of 11 years (range, 2 to 45 years). The majority of subjects (n = 28) have type 3 vWD. The mean dose used for treatment is 24 units factor VIII/kg body weight given one to three times weekly. Indications for prophylaxis have included joint bleeds, bleeds from nose and mouth, menorrhagia and gastrointestinal bleeds. The annual number of bleeds has decreased dramatically following onset of prophylaxis. We conclude that long-term prophylactic treatment of vWD is warranted in the majority of cases with type 3 and in some cases, depending on the clinical phenotype, for patients with other subtypes. Additional studies are ongoing in an international effort, the von Willebrand Disease Prophylaxis Network. (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
prophylaxis, bleeding, VIII, factor, von Willebrand factor concentrates, von Willebrand disease
in
Seminars in Thrombosis and Hemostasis
volume
32
issue
6
pages
621 - 625
publisher
Georg Thieme Verlag KG
external identifiers
  • wos:000240864100008
  • scopus:33749027736
ISSN
1098-9064
DOI
10.1055/s-2006-949667
language
English
LU publication?
yes
id
17dabf81-1515-4909-b704-43ec39b8278f (old id 160958)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16977572&dopt=Abstract
date added to LUP
2016-04-01 16:21:40
date last changed
2020-01-12 19:17:55
@article{17dabf81-1515-4909-b704-43ec39b8278f,
  abstract     = {Treatment of type 3 von Willebrand disease (vWD) relies on infusion with plasma-derived factor concentrates containing von Willebrand factor (vWF). Patients with types 1 and 2 vWD who do not respond satisfactorily after receiving desmopressin need treatment with concentrates. The rationale for long-term prophylaxis in vWD is obvious: prophylaxis has been successfully used in hemophilia, and joint hemorrhages with development of hemophilic arthropathy can occur, especially in type 3 vWD. In Sweden, prophylaxis for vWD began during the 1960s, and we now have experience from a cohort of 37 patients treated for a median of 11 years (range, 2 to 45 years). The majority of subjects (n = 28) have type 3 vWD. The mean dose used for treatment is 24 units factor VIII/kg body weight given one to three times weekly. Indications for prophylaxis have included joint bleeds, bleeds from nose and mouth, menorrhagia and gastrointestinal bleeds. The annual number of bleeds has decreased dramatically following onset of prophylaxis. We conclude that long-term prophylactic treatment of vWD is warranted in the majority of cases with type 3 and in some cases, depending on the clinical phenotype, for patients with other subtypes. Additional studies are ongoing in an international effort, the von Willebrand Disease Prophylaxis Network.},
  author       = {Berntorp, Erik},
  issn         = {1098-9064},
  language     = {eng},
  number       = {6},
  pages        = {621--625},
  publisher    = {Georg Thieme Verlag KG},
  series       = {Seminars in Thrombosis and Hemostasis},
  title        = {Prophylaxis and treatment of bleeding complications in von Willebrand disease type 3.},
  url          = {http://dx.doi.org/10.1055/s-2006-949667},
  doi          = {10.1055/s-2006-949667},
  volume       = {32},
  year         = {2006},
}