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The impact of bleed...
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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Castaman, Giancarlo (author)
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Tosetto, Alberto (author)
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Goodeve, Anne (author)
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Federici, Augusto B. (author)
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- Lethagen, Stefan (author)
- Lund University,Lunds universitet,Institutionen för kliniska vetenskaper, Malmö,Medicinska fakulteten,Department of Clinical Sciences, Malmö,Faculty of Medicine
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Budde, Ulrich (author)
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Batlle, Javier (author)
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Meyer, Dominique (author)
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Mazurier, Claudine (author)
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Goudemand, Jenny (author)
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Eikenboom, Jeroen (author)
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Schneppenheim, Reinhard (author)
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Ingerslev, Jorgen (author)
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Habart, David (author)
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Hill, Frank (author)
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Peake, Ian (author)
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Rodeghiero, Francesco (author)
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(creator_code:org_t)
- 2010-08-25
- 2010
- English.
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In: British Journal of Haematology. - : Wiley. - 0007-1048. ; 151:3, s. 245-251
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Abstract
Subject headings
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- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Keyword
- bleeding score
- PFA-100 closure time
- disorders
- inherited bleeding
- von Willebrand disease
- von Willebrand factor
Publication and Content Type
- art (subject category)
- ref (subject category)
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- By the author/editor
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Castaman, Gianca ...
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Tosetto, Alberto
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Goodeve, Anne
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Federici, August ...
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Lethagen, Stefan
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Budde, Ulrich
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show more...
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Batlle, Javier
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Meyer, Dominique
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Mazurier, Claudi ...
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Goudemand, Jenny
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Eikenboom, Jeroe ...
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Schneppenheim, R ...
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Ingerslev, Jorge ...
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Habart, David
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Hill, Frank
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Peake, Ian
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Rodeghiero, Fran ...
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show less...
- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Hematology
- Articles in the publication
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British Journal ...
- By the university
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Lund University