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Clin Appl Thromb Hemost. 2014 Nov;20(8):765-71. doi: 10.1177/1076029614543825. Epub 2014 Jul 25.

Systematic analysis of bleeding phenotype in PT-VWD compared to type 2B VWD using an electronic bleeding questionnaire.

Author information

1
Department of Biomedical and Molecular Sciences, Queen's University, Kingston, Ontario, Canada.
2
Faculty of Medical Sciences, University of Campinas, Sao Paulo, Brazil.
3
New Atom Technologies Inc, Kingston, Ontario, Canada.
4
Department of Pathology and Molecular Medicine, Queen's University, Kingston, Ontario, Canada.
5
Department of Biomedical and Molecular Sciences, Queen's University, Kingston, Ontario, Canada School of Baccalaureate Nursing, St Lawrence College, Kingston, Ontario, Canada othman@queensu.ca.

Abstract

OBJECTIVE:

To investigate the utility of an electronic version of the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease (VWD) bleeding questionnaire (eBQ) in assessing the bleeding phenotype in platelet-type VWD (PT-VWD) and compare it to its closely similar disorder, type 2B VWD.

METHODS:

Retrospective analysis of the clinical bleeding and laboratory phenotype of 13 patients with PT-VWD and 12 type 2B VWD.

RESULTS:

Bleeding score (BS) was significantly lower in PT-VWD as compared to type 2B. Bleeding score correlated with platelet count and von Willebrand factor:Ristocetin cofactor activity in PT-VWD but not in type 2B with a significant reduction in platelet count in type 2B VWD compared to PT-VWD. The eBQ had sensitivity of 62% in PT-VWD and 92% in type 2B VWD.

CONCLUSION:

Objective analysis of bleeding symptoms further the understanding of the phenotype of 2 closely similar bleeding disorders for better diagnosis and follow-up. Larger international prospective studies are warranted to evaluate the utility of the eBQ in PT-VWD and other rare bleeding disorders.

KEYWORDS:

glycoproteins; platelet dysfunction; platelets; thrombocytopenia; von Willebrand disease

PMID:
25063765
DOI:
10.1177/1076029614543825
[Indexed for MEDLINE]

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