title: Systematic analysis of bleeding phenotype in PT-VWD compared to type 2B VWD using an electronic bleeding questionnaire.
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.
  
  
  CONCLUSIONS
  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.
authors: Department of Biomedical and Molecular Sciences, Queen's University, Kingston, Ontario, Canada.
authors: Harmanpreet
authors: H
authors: Kaur
authors: Faculty of Medical Sciences, University of Campinas, Sao Paulo, Brazil.
authors: Margareth
authors: M
authors: Ozelo
authors: New Atom Technologies Inc, Kingston, Ontario, Canada.
authors: Stephen
authors: S
authors: Scovil
authors: Department of Pathology and Molecular Medicine, Queen's University, Kingston, Ontario, Canada.
authors: Paula D
authors: PD
authors: James
authors: 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.
authors: Maha
authors: M
authors: Othman
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D006470:Hemorrhage / Q000097:blood / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D056728:von Willebrand Disease, Type 2 / Q000097:blood / Q000175:diagnosis*; D014842:von Willebrand Diseases / Q000097:blood / Q000175:diagnosis*
keywords: glycoproteins; platelet dysfunction; platelets; thrombocytopenia; von Willebrand disease