Clinical experience of prophylactic treatment in von Willebrand disease

The prophylactic treatment of bleeding in von Willebrand disease (vWD) has a long history in Sweden, having been first initiated in the late 1950s. The clinical experience of the prophylaxis of vWD in Sweden is described in the current review of a retrospective study of 37 patients from three haemophilia centres receiving prophylaxis for vWD. Prophylaxis with a plasma concentrate (fraction I-0 or Factor VIII) effectively reduced the median number of bleeds per year, prevented joint arthropathy in those who started prophylaxis at a young age, and improved the quality of life of all patients with vWD, particularly those with type 3 disease. This study demonstrates that the long-term prophylactic treatment with von Willebrand Factor (vWF)/FVIII concentrate (Haemate-P, ZLB Behring) in vWD is warranted in the majority of patients with type 3 disease.