Standard thromboprophylaxis should be used in patients in whom VWF levels are normalized. Over the past decade, it has become clear that in severe forms of VWD, long-term prophylaxis is beneficial [21-23]. As mucosal surfaces are rich in fibrinolytic activity [9], blocking fibrinolysis is a useful adjunctive measure to stop bleeding. Epsilon-aminocaproic acid (at a dose

of 50–60 mg kg−1 every 4–6 h) or tranexamic acid (at a dose of 10–15 mg kg−1 every 8–12 h) may be administered orally, intravenously, or topically [9]. Oestrogen–progesteron preparations render the endometrium less susceptible to bleeding, and may be very useful in managing CHIR-99021 in vitro menorrhagia in VWD patients [8, 9]. As there are no population-based data, the prevalence of inherited platelet disorders, which encompass both functional disorders and thrombocytopenia (Table 3), remains unknown. In studies of SCH727965 price patients presenting with mucocutaneous bleeding, platelet abnormalities are at least as common as VWD. Severe disorders are often recognized in childhood, but mild disorders may go undiagnosed unless there is a family history that prompts testing, or until a haemostatic challenge results in significant bleeding. Algorithms have been developed to aid with the investigation

of inherited platelet function disorders [24] (available at: www.ahcdc.ca/index.php/research/rare-inherited-bleeding-disorders) [25], and thrombocytopenias [26]. Validated bleeding assessment tools (BATs) are useful

in standardizing information obtained learn more from the patient history and accurately recording the severity and frequency of bleeding symptoms [27]. The high negative predictive value of some of these tools may make it possible to use them as a screen prior to laboratory testing. However, existing tools have low specificity and will not provide a definitive diagnosis [27, 28]. There is no ideal simple, inexpensive, sensitive screening test that reliably identifies patients requiring specialized testing of platelet function. Although both bleeding times and PFA-100/200® closure times have been used for this purpose, these tests are not adequately sensitive to rule out the need for further testing [29], and should be considered optional. A validated BAT may be more useful in assessing a patient’s bleeding propensity and determining whether further specialized laboratory investigations are warranted. The most widely used method for assessing platelet function is light transmission aggregometry (LTA), in which the change in optical density of a stirred sample of citrated platelet-rich plasma is measured by a photometer following the addition of agonists. Although many pre-analytical and analytical variables affect the results, and international surveys have shown that there is wide variation in methodology, LTA remains the gold standard platelet function test. Recommendations for standardization have recently been published [30].