Von Willebrand disease

We recommend DDAVP as a first-line treatment for minor bleeding/surgery in patients with von Willebrand disease (VWD), after a trial testing. The standard regimen is 0.3 mgkg-1 dissolved in 50 ml saline and infused IV over 20 to 30 min, repeated every 12 to 24 h usually for no more than 3 days. 1C

We recommend replacement of von Willebrand factor (VWF) with plasma-derived products for major bleeding/ surgery. Treatment regimens are specified by published guidelines. 1C

We suggest that antifibrinolytic drugs be used as haemostatic adjuncts. Treatment regimens are specified by published guidelines. 2C