Different bleeding risk in type 2A and 2M von Willebrand disease: A 2-year prospective study in 107 patients

G. Castaman, A. B. Federici, A. Tosetto, S. La Marca, F. Stufano, P. M. Mannucci, F. Rodeghiero

Research output: Contribution to journalArticle

Abstract

Background: Type 2A and 2M von Willebrand disease (VWD2A and VWD2M) are characterized by the presence of a dysfunctional von Willebrand factor (VWF) and a variable bleeding tendency. So far, a head-to-head comparison of the clinical history and bleeding risk between VWD2A and VWD2M has never been provided in a prospective manner. Aim of the study: We assessed the bleeding incidence rate and clinical characteristics in two cohorts of 17 families (46 patients) with VWD2A and 15 families (61 patients) with VWD2M prospectively followed-up for 24months. VWF gene mutations were characterized in all of them. Results: Mean bleeding score (BS) and VWF antigen at enrollment were significantly higher in VWD2A patients (P=0.007). No correlation between VWF activity or factor VIII levels and the severity of BS was observed. The incidence rate of spontaneous bleeding requiring treatment was 107/100 patient-years (95% CI, 88.3-131) in VWD2A compared with 40/100 patient-years (95% CI, 30-53) in VWD2M (P

Original languageEnglish
Pages (from-to)632-638
Number of pages7
JournalJournal of Thrombosis and Haemostasis
Volume10
Issue number4
DOIs
Publication statusPublished - Apr 2012

Keywords

  • Gene mutation
  • Inherited bleeding disorder
  • Von Willebrand disease
  • Von Willebrand factor

ASJC Scopus subject areas

  • Hematology

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