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

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.

Castaman, G (författare)
Tosetto, A (författare)
Batlle, J (författare)
Baudo, F (författare)
Cappelletti, A (författare)
Casana, P (författare)
De Bosch, N (författare)
Eikenboom, JCJ (författare)
Federici, AB (författare)
Lethagen, StefanLund University,Lunds universitet,Institutionen för kliniska vetenskaper, Malmö,Medicinska fakulteten,Department of Clinical Sciences, Malmö,Faculty of Medicine(Swepub:lu)medf-sle (författare)
Linari, S (författare)
Srivastava, A (författare)
Institutionen för kliniska vetenskaper, MalmöMedicinska fakulteten (creator_code:org_t)

Ingår i:Journal of Thrombosis and Haemostasis: Elsevier BV3:12, s. 2619-26261538-79331538-7836

Av författaren/redakt...: Rodeghiero, F; Castaman, G; Tosetto, A; Batlle, J; Baudo, F; Cappelletti, A; visa fler...; Casana, P; De Bosch, N; Eikenboom, JCJ; Federici, AB; Lethagen, Stefan; Linari, S; Srivastava, A; visa färre...

Om ämnet

MEDICIN OCH HÄLSOVETENSKAP: MEDICIN OCH HÄLS ...; och Klinisk medicin; och Kardiologi

Kungliga biblioteket hanterar dina personuppgifter i enlighet med EU:s dataskyddsförordning (2018), GDPR. Läs mer om hur det funkar här.
Så här hanterar KB dina uppgifter vid användning av denna tjänst.