Assessing bleeding in von Willebrand disease with bleeding score

Quantification of the bleeding severity in von Willebrand disease (VWD) by use of a bleeding score (BS) could be a useful tool toward a more objective diagnosis. The BS is computed as the sum of arbitrary grades of bleeding severity for several hemorrhagic symptoms, and in normal subjects is nearly always lower than 3. In the diagnosis of type 1 VWD, the use of the BS provided a high specificity (99.1%) and a satisfactory sensitivity (64.2%) in a multicenter, retrospective study. The BS is strongly related to all available measurements of von Willebrand factor activity, including the PFA-100 closure time. The BS could be used to assess the influence on bleeding symptoms of other biological factors in VWD. Therefore, BS represent a promising clinical tool for the analysis of bleeding in VWD, although further validation is warranted before an extensive use in clinical practice could be purported.