Although some of these influences are well documented (i.e. ABO blood group), others have either only very recently been detected or remain to be characterized. A personal history of excessive mucocutaneous bleeding is a key component in the diagnosis of a number Gefitinib mw of mild bleeding disorders,

including VWD, platelet function disorders and coagulation factor deficiencies. However, the evaluation of haemorrhagic symptoms is a well recognized challenge for both patients and physicians, because the reporting and interpretation of bleeding symptoms is subjective. As a result, bleeding assessment tools (BATs) have been developed and studied in a variety of clinical settings [34, 52, 53]. These tools are invaluable in the identification of symptomatic patients; symptomatic XL765 manufacturer VWD has a prevalence of ∼1 in 1000; however, a review of diagnosed cases reveals that far fewer patients have been diagnosed, and therefore, far fewer have access to appropriate treatment. The work in BATs has been pioneered by a group of Italian researchers, and the resultant ‘Vicenza Bleeding Questionnaire’ stands as the original BAT. Over the years various modifications of the Vicenza Bleeding Questionnaire have taken place (Fig. 6), and validation studies have been published. The Condensed MDMCM–1 VWD Bleeding Questionnaire is the one developed and validated by the group

at Queen’s University, Kingston, Ontario, Canada [53]. It is a summative standardized scoring system, which was condensed from the MCMDM–1VWD version by removing all questions that do not directly affect the bleeding score. It has been validated prospectively as a screening tool for VWD with a sensitivity of 100%, specificity of 87%, positive predictive value of 0.20 and negative predictive

value of 1.0. A subsequent, paediatric-specific version has also been developed and validated, although given the lack of haemostatic challenges in children, it is less sensitive compared with adults [54]. The ISTH–BAT was published in 2010 [55], Sitaxentan which builds on the knowledge of the previous Vicenza-based BATs with some important modifications; it captures the frequency of bleeding episodes in contrast to the previous version, which saturates if used in more severe bleeding disorders. A web-based version is available through Rockefeller University [56]. Overall, BATs can distinguish between normal and persons with a bleeding disorder, identify those at risk of having a bleeding disorder, those who are very unlikely to have a bleeding disorder and describe disease severity within a limited spectrum. BATs are limited in the setting of menorrhagia, because no assessment of quality of life is included, and heavy menstrual bleeding has such a negative affect on quality of life.