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.
© The Author(s) 2014.