Lethagen Stefan
Copenhagen Hemophilia Center, Rigshospitalet, Afs. 5002, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.
Thromb Res. 2006;118 Suppl 1:S9-11. doi: 10.1016/j.thromres.2006.01.021. Epub 2006 Apr 17.
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.
在瑞典，血管性血友病（vWD）出血的预防性治疗已有很长历史，始于20世纪50年代末。本文对来自三个血友病中心的37例接受vWD预防性治疗的患者进行回顾性研究，描述了瑞典vWD预防性治疗的临床经验。使用血浆浓缩物（I-0组分或凝血因子VIII）进行预防可有效降低每年出血的中位数，预防了幼年开始预防的患者的关节病，并改善了所有vWD患者的生活质量，尤其是3型疾病患者。这项研究表明，大多数3型疾病患者有必要长期使用血管性血友病因子（vWF）/FVIII浓缩物（海莫特-P，杰特贝林）进行预防性治疗。
