The management of patients with congenital von Willebrand disease during surgery or other invasive procedures: focus on antihemophilic factor/von Willebrand factor complex

Von Willebrand disease, the most common hereditary bleeding disorder, arises from quantitative or qualitative defect of von Willebrand factor (VWF). The aim of the treatment is to correct the dual defect of hemostasis caused by the abnormal/reduced VWF and the concomitant deficiency of factor VIII (FVIII). The synthetic vasopressin analogue desmopressin is the mainstay of therapy in about 80% of patients, while nearly 20% are unresponsive and must be treated with FVIII/VWF concentrates. This latter therapeutic option will be focused in the review, with particular consideration to the management of surgery and invasive procedures in these patients.