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J Thromb Haemost. 2005 Dec;3(12):2619-26.

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

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1
Hematology Department, S. Bortolo Hospital, Vicenza, Italy. rodeghiero@hemato.ven.it

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  • J Thromb Haemost. 2006 Apr;4(4):925.

Abstract

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.

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