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
(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)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/1721017
- author
- organization
- publishing date
- 2010
- type
- Contribution to journal
- publication status
- published
- subject
- 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
- Wiley-Blackwell
- external identifiers
-
- wos:000283069100005
- scopus:79952049046
- pmid:20738304
- ISSN
- 0007-1048
- DOI
- 10.1111/j.1365-2141.2010.08333.x
- language
- English
- LU publication?
- yes
- additional info
- 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)
- id
- 8c45b0a6-b880-4d18-aef8-fc0cb5fc7eba (old id 1721017)
- date added to LUP
- 2016-04-01 10:53:48
- date last changed
- 2022-08-12 23:57:59
@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}},
keywords = {{bleeding score; PFA-100 closure time; disorders; inherited bleeding; von Willebrand disease; von Willebrand factor}},
language = {{eng}},
number = {{3}},
pages = {{245--251}},
publisher = {{Wiley-Blackwell}},
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}},
doi = {{10.1111/j.1365-2141.2010.08333.x}},
volume = {{151}},
year = {{2010}},
}