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

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

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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Child, Preschool
  • Female
  • Hemorrhage / blood
  • Hemorrhage / diagnosis*
  • Humans
  • Male
  • Middle Aged
  • Phenotype
  • Severity of Illness Index
  • Surveys and Questionnaires
  • von Willebrand Disease, Type 2 / blood
  • von Willebrand Disease, Type 2 / diagnosis*
  • von Willebrand Diseases / blood
  • von Willebrand Diseases / diagnosis*

Supplementary concepts

  • Von Willebrand disease, platelet type