Von Willebrand disease Management Desmopressin trial BSH Q Is vWD type 1, 2A, 2M or 2N present? Q Is known atherosclerosis absent? Yes to all Perform a trial of desmopressin in patients with type 1, 2A, 2M, and 2N vWD. Measure vWF antigen, activity and factor VIII at baseline, 30-60 minutes, and 4-6 hours. B Advise adults to limit fluid intake to 1L in the first 24h after desmopressin. B Avoid desmopressin in patients with known atherosclerosis. D Desmopressin trial BSH Q Is desmopressin trial positive? No If the patient is non-desmopressin responsive, then acute treatment and secondary prophylaxis using concentrates should be considered. Yes Use desmopressin in preference to blood-derived products, whenever possible (e.g., minor bleed or surgery), in patients in whom desmopressin has previously been shown to be effective. B No to any Management of acute bleeding Q Does the patient have acute bleeding? Q Does the patient need surgery? Yes to any Administer a vWF-FVIII concentrate or a combination of high purity FVIII and high purity vWF concentrates for treatment of acute bleeding or emergency surgery. A Use desmopressin in preference to blood-derived products, whenever possible (e.g., minor bleed or surgery), if previously shown to be effective. B No to all Prevention of recurrent bleeding BSH Q Does the patient have recurrent bleeding? Yes Consider initiating prophylaxis in patients with vWD of any type in whom there is recurrent bleeding. B Use intermediate purity FVIII-VWF or high purity VWF concentrates for prophylaxis. B No Continue management as clinically appropriate.