A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD).

摘要: Background: A quantitative description of bleeding symptoms in type 1 von Willebrand disease (VWD) has never been reported. Objectives: The aim was to quantitatively evaluate the severity VWD and its correlation with clinical laboratory features. Patients methods: Bleeding were retrospectively recorded a European cohort families, for each subject score (BS) obtained together phenotypic tests. Results: total 712 subjects belonging 144 families 195 controls available analysis. BS higher index cases than affected family members (BS 9 vs. 5, P < 0.0001) unaffected 0 -1, 0.0001). There no effect ABO blood group. showed strong significant inverse relation either ristocetin cofactor (VWF:RCo), antigen (VWF:Ag) or factor VIII procoagulant activity (FVIII:C) measured at time enrollment, even after adjustment age, sex group (P 0.001 all four upper quintiles first quintile, VWF:RCo, VWF:Ag FVIII:C). Higher related increasing likelihood VWD, mucocutaneous (computed from spontaneous, symptoms) strongly associated surgery tooth extraction. Conclusions: Quantitative analysis is potentially useful more accurate diagnosis develop guidelines optimal treatment. (Less)