Mediterranean Journal of Hematology and Infectious Diseases

Quantification of the bleeding severity by use of bleeding assessment tools (BAT) and bleeding score (BS) has been consistently shown to improve the clinical diagnosis of von Willebr disease (VWD) while helping researchers establish phenotype/genotype correlations. Subjects with a BS equal or higher than 3 may be consider having a bleeding tendency, and should be referred for a laboratory investigation, particularly for VWD. In th BS has been shown to be highly specific (>95%) with reported sensitivities ranging from 40 to 100%. The BS is related to all available measurements of von Willebrand factor activity, including the PFA-100 closure time. Therefore, in clinical practice the use of BAT should always be the first step to standardize the assessment of patients with suspected VWD. The use of the recent ISTH consensus BAT is suggested to harmonize the collection of bleeding symptoms in pati suspected or confirmed hemostatic disorder, particularly VWD. The ISTH BAT is also coupled with a Web-based repository of bleeding symptoms, therefore providing an integrated framework for collaboration in the field of clinical evaluation of VW Introduction. The diagnosis of von Willebrand disease (VWD) may be consider as the epitome of the difficulties encountered in the diagnosis of mild bleeding disorders. In contrast with patients having a severe bleeding disorder, who usually have a clear clinical and laboratory phenotype, VWD patients usually report a mild bleeding history and have a partial reduction of plasma von Willebrand factor levels. This clinical and laboratory pattern is consistent with the heterozygous transmission of the defect, with circulating von Willebrand factor (VWF) around 30 IU/dL without evident structural abnormalities in the www.mjhid.org ISSN 2035-3006 Clinical Markers for the Correct Diagnosis of , S. Bortolo Hospital, via Rodolfi 37, 36100 Vicenza, . E-mail: [email protected] at no competing interests exist. (1): e2013051, DOI: 10.4084/MJHID.2013.051 rmits unrestricted use, distribution, and reproduction in any medium, e diagnosis of type 1 VWD, the use of the D and mild bleeding disorders.