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

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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; Budde, Ulrich; Batlle, Javier; Meyer, Dominique; Mazurier, Claudine; Goudemand, Jenny; Eikenboom, Jeroen; Schneppenheim, Reinhard; Ingerslev, Jorgen; Habart, David; Hill, Frank; Peake, Ian; Rodeghiero, Francesco.

I: British Journal of Haematology, Vol. 151, Nr. 3, 2010, s. 245-251.

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskrift

Harvard

Castaman, G, Tosetto, A, Goodeve, A, Federici, AB, Lethagen, S, Budde, U, Batlle, J, Meyer, D, Mazurier, C, Goudemand, J, Eikenboom, J, Schneppenheim, R, Ingerslev, J, Habart, D, Hill, F, Peake, I & Rodeghiero, F 2010, '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', British Journal of Haematology, vol. 151, nr. 3, s. 245-251. https://doi.org/10.1111/j.1365-2141.2010.08333.x

APA

CBE

Castaman G, Tosetto A, Goodeve A, Federici AB, Lethagen S, Budde U, Batlle J, Meyer D, Mazurier C, Goudemand J, Eikenboom J, Schneppenheim R, Ingerslev J, Habart D, Hill F, Peake I, Rodeghiero F. 2010. 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. British Journal of Haematology. 151(3):245-251. https://doi.org/10.1111/j.1365-2141.2010.08333.x

MLA

Vancouver

Author

Castaman, Giancarlo ; Tosetto, Alberto ; Goodeve, Anne ; Federici, Augusto B. ; Lethagen, Stefan ; Budde, Ulrich ; Batlle, Javier ; Meyer, Dominique ; Mazurier, Claudine ; Goudemand, Jenny ; Eikenboom, Jeroen ; Schneppenheim, Reinhard ; Ingerslev, Jorgen ; Habart, David ; Hill, Frank ; Peake, Ian ; Rodeghiero, Francesco. / 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. I: British Journal of Haematology. 2010 ; Vol. 151, Nr. 3. s. 245-251.

RIS

TY - JOUR

T1 - 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

AU - Castaman, Giancarlo

AU - Tosetto, Alberto

AU - Goodeve, Anne

AU - Federici, Augusto B.

AU - Lethagen, Stefan

AU - Budde, Ulrich

AU - Batlle, Javier

AU - Meyer, Dominique

AU - Mazurier, Claudine

AU - Goudemand, Jenny

AU - Eikenboom, Jeroen

AU - Schneppenheim, Reinhard

AU - Ingerslev, Jorgen

AU - Habart, David

AU - Hill, Frank

AU - Peake, Ian

AU - Rodeghiero, Francesco

N1 - 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)

PY - 2010

Y1 - 2010

N2 - 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.

AB - 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.

KW - bleeding score

KW - PFA-100 closure time

KW - disorders

KW - inherited bleeding

KW - von Willebrand disease

KW - von Willebrand factor

U2 - 10.1111/j.1365-2141.2010.08333.x

DO - 10.1111/j.1365-2141.2010.08333.x

M3 - Article

VL - 151

SP - 245

EP - 251

JO - British Journal of Haematology

JF - British Journal of Haematology

SN - 0007-1048

IS - 3

ER -