Contemporary issues in the management of von Willebrand disease

Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Bleeding scores in VWD, focused in particular on mucosal bleeding, can be very useful in the diagnosis and validation of different types of treatment. The results of an extended prospective study with a large amount of information on clinical phenotype and implications in treatment are reviewed in this article. Treatment of mucosal and joint bleeding in severe VWD remains difficult in some patients. Due to the lack of data on the use of prophylaxis in these patients it is difficult to establish optimal treatment regimens. An overview of the literature, with a focus on the ongoing PRO.WILL study, is provided here. Furthermore, understanding the changes in von Willebrand factor (VWF) levels during pregnancy is very important for establishing the optimal management strategy for pregnancy and delivery in women with VWD. A recently published prospective observational cohort study in women with and without VWD during the postpartum period provides important data that should allow the improvement of postpartum treatment protocols.