von Willebrand disease: An update on diagnosis and treatment

von Willebrand disease (VWD) is the most common congenital bleeding disorder with a potential incidence of 1% of the general population. The disorder is classified into three main types: type 1 and type 3 as (respectively, partial and complete) quantitative deficiency of von Willebrand factor (VWF) and type 2 (with qualitative VWF defects). The bleeding tendency is highly variable, ranging from largely asymptomatic, mainly in mild type 1 VWD, to severe life-threatening hemorrhage, notably type 3 VWD. Diagnosis of VWD requires comprehensive laboratory testing aligned to appropriate clinical assessment. On-demand therapy is the mainstay of treatment in VWD, although long-term prophylaxis is emerging for those with recurrent and severe bleeding. Therapeutic choices in VWD depend to some extent on geographical location, but include VWF/FVIII concentrates, recombinant VWF, and desmopressin.