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The impact of bleeding history, von Willebrand factor and PFA-100 (R) on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM-1VWD

Castaman, Giancarlo; Tosetto, Alberto; Goodeve, Anne; Federici, Augusto B.; Lethagen, Stefan LU ; Budde, Ulrich; Batlle, Javier; Meyer, Dominique; Mazurier, Claudine and Goudemand, Jenny, et al. (2010) In British Journal of Haematology 151(3). p.245-251
Abstract
P>The relationships between the Platelet Function Analyzer (PFA)-100 and von Willebrand factor (VWF) levels and bleeding score (BS) were evaluated within a multicentre project on Molecular and Clinical Markers for the Diagnosis and Management of type 1 von Willebrand disease (MCMDM-1VWD). PFA-100 closure time, either with epinephrine (EPI) or adenosine diphosphate (ADP)-cartridges, was measured in 107 index cases, 105 affected and 71 unaffected family members, and 79 healthy controls. By regression analysis VWF levels were strongly related to both closure times, with a non-linear progression. In a multiple stepwise regression model, age- and sex-adjusted PFA-100 ADP and VWF ristocetin cofactor activity (VWF:RCo) were independently... (More)
P>The relationships between the Platelet Function Analyzer (PFA)-100 and von Willebrand factor (VWF) levels and bleeding score (BS) were evaluated within a multicentre project on Molecular and Clinical Markers for the Diagnosis and Management of type 1 von Willebrand disease (MCMDM-1VWD). PFA-100 closure time, either with epinephrine (EPI) or adenosine diphosphate (ADP)-cartridges, was measured in 107 index cases, 105 affected and 71 unaffected family members, and 79 healthy controls. By regression analysis VWF levels were strongly related to both closure times, with a non-linear progression. In a multiple stepwise regression model, age- and sex-adjusted PFA-100 ADP and VWF ristocetin cofactor activity (VWF:RCo) were independently associated with BS. Most of the variation of BS was predicted by PFA-100 ADP and VWF:RCo alone. In the subgroup of patients with subtle abnormalities of the multimeric pattern, VWF was invariably reduced and closure time prolonged in almost all of them. Neither PFA-100 ADP nor EPI closure times appeared to significantly improve the diagnostic capability of VWF antigen (VWF:Ag) measurement. Thus, in an unselected population a normal PFA-100 would be useful to exclude VWD, but whether it could replace the more specific VWF assay in patients with significant mucocutaneous bleeding symptoms remains to be investigated prospectively. (Less)
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published
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keywords
bleeding score, PFA-100 closure time, disorders, inherited bleeding, von Willebrand disease, von Willebrand factor
in
British Journal of Haematology
volume
151
issue
3
pages
245 - 251
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000283069100005
  • scopus:79952049046
ISSN
0007-1048
DOI
10.1111/j.1365-2141.2010.08333.x
language
English
LU publication?
yes
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8c45b0a6-b880-4d18-aef8-fc0cb5fc7eba (old id 1721017)
date added to LUP
2010-12-03 12:46:59
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2018-06-10 03:27:39
@article{8c45b0a6-b880-4d18-aef8-fc0cb5fc7eba,
  abstract     = {P>The relationships between the Platelet Function Analyzer (PFA)-100 and von Willebrand factor (VWF) levels and bleeding score (BS) were evaluated within a multicentre project on Molecular and Clinical Markers for the Diagnosis and Management of type 1 von Willebrand disease (MCMDM-1VWD). PFA-100 closure time, either with epinephrine (EPI) or adenosine diphosphate (ADP)-cartridges, was measured in 107 index cases, 105 affected and 71 unaffected family members, and 79 healthy controls. By regression analysis VWF levels were strongly related to both closure times, with a non-linear progression. In a multiple stepwise regression model, age- and sex-adjusted PFA-100 ADP and VWF ristocetin cofactor activity (VWF:RCo) were independently associated with BS. Most of the variation of BS was predicted by PFA-100 ADP and VWF:RCo alone. In the subgroup of patients with subtle abnormalities of the multimeric pattern, VWF was invariably reduced and closure time prolonged in almost all of them. Neither PFA-100 ADP nor EPI closure times appeared to significantly improve the diagnostic capability of VWF antigen (VWF:Ag) measurement. Thus, in an unselected population a normal PFA-100 would be useful to exclude VWD, but whether it could replace the more specific VWF assay in patients with significant mucocutaneous bleeding symptoms remains to be investigated prospectively.},
  author       = {Castaman, Giancarlo and Tosetto, Alberto and Goodeve, Anne and Federici, Augusto B. and Lethagen, Stefan and Budde, Ulrich and Batlle, Javier and Meyer, Dominique and Mazurier, Claudine and Goudemand, Jenny and Eikenboom, Jeroen and Schneppenheim, Reinhard and Ingerslev, Jorgen and Habart, David and Hill, Frank and Peake, Ian and Rodeghiero, Francesco},
  issn         = {0007-1048},
  keyword      = {bleeding score,PFA-100 closure time,disorders,inherited bleeding,von Willebrand disease,von Willebrand factor},
  language     = {eng},
  number       = {3},
  pages        = {245--251},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {British Journal of Haematology},
  title        = {The impact of bleeding history, von Willebrand factor and PFA-100 (R) on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM-1VWD},
  url          = {http://dx.doi.org/10.1111/j.1365-2141.2010.08333.x},
  volume       = {151},
  year         = {2010},
}