title: Clinical experience of prophylactic treatment in von Willebrand disease.
abstract: 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.
authors.affiliation: Copenhagen Hemophilia Center, Rigshospitalet, Afs. 5002, Blegdamsvej 9, DK-2100 Copenhagen, Denmark. stefan.lethagen@rh.dk
authors.forename: Stefan
authors.initials: S
authors.lastname: Lethagen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002985:Clinical Protocols; D005169:Factor VIII / Q000627:therapeutic use*; D005260:Female; D006395:Hemarthrosis / Q000209:etiology / Q000517:prevention & control; D006470:Hemorrhage / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D008297:Male; D008875:Middle Aged; D011292:Premedication; D011788:Quality of Life; D012189:Retrospective Studies; D013548:Sweden; D014842:von Willebrand Diseases / Q000150:complications / Q000188:drug therapy*
chemical_list: D005169:Factor VIII