The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study

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Objective: The aim of this study was the validation of the criteria defining a significant mucocutaneous-bleeding history in type 1 von Willebrand disease (VWD). Subjects and methods: To avoid selection bias, 42 obligatory carriers (OC) of type 1 VWD were identified from a panel of 42 families with type 1 VWD enrolled by 10 expert centers. OC were identified by the presence of an offspring and another first degree relative with type 1 VWD (affected subjects, AFF). A standardized questionnaire was administered to evaluate hemorrhagic symptoms at the time of first examination, using a bleeding score ranging from 0 (no symptom) to 3 (hospitalization, replacement therapy, blood transfusion). Sensitivity, specificity, diagnostic likelihood ratios, positive and negative predictive values for the diagnosis of type 1 VWD were calculated from the data collected in OC and in 215 controls. Results: Having at least three hemorrhagic symptoms or a bleeding score of 3 in males and 5 in females was very specific (98.6%) for the bleeding history of type 1 VWD, although less sensitive (69.1%). None of the misclassified OC had life-threatening bleeding episodes after diagnosis. Conclusions: We suggest that the use of a standardized questionnaire and bleeding score may be useful for the identification of subjects requiring laboratory evaluation for VWD.


  • F Rodeghiero
  • G Castaman
  • A Tosetto
  • J Batlle
  • F Baudo
  • A Cappelletti
  • P Casana
  • N De Bosch
  • JCJ Eikenboom
  • AB Federici
  • Stefan Lethagen
  • S Linari
  • A Srivastava
Enheter & grupper

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Kardiologi


Sidor (från-till)2619-2626
TidskriftJournal of Thrombosis and Haemostasis
StatusPublished - 2005
Peer review utfördJa