Mannucci P M
Department of Internal Medicine, IRCCS Maggiore Hospital, University of Milan, Italy.
Thromb Haemost. 2001 Jul;86(1):149-53.
In von Willebrand disease, there are two main options for the treatment of spontaneous bleeding episodes and for bleeding prophylaxis: desmopressin and transfusional therapy with plasma products. Desmopressin is the treatment of choice for most patients with type 1, who account for approximately 70 to 80 per cent of all cases with the disease. This non-transfusional hemostatic agent raises endogenous factor VIII and von Willebrand factor three- to fivefold and thereby transiently corrects both the intrinsic coagulation and primary hemostasis defects. In patients with the more severe type 3 and in the majority of those with type 2 desmopressin is not effective or is contraindicated, so that it is usually necessary to resort to plasma concentrates containing factor VIII and von Willebrand factor. Concentrates treated with virus inactivation methods should be preferred to cryoprecipitate because they are equally effective and perceived as safer.
在血管性血友病中，治疗自发性出血发作和预防出血主要有两种选择：去氨加压素和使用血浆制品的输血治疗。去氨加压素是大多数1型患者的首选治疗方法，1型患者约占该病所有病例的70%至80%。这种非输血性止血剂可使内源性因子VIII和血管性血友病因子升高三至五倍，从而暂时纠正内源性凝血和初级止血缺陷。对于更严重的3型患者以及大多数2型患者，去氨加压素无效或禁忌使用，因此通常需要使用含有因子VIII和血管性血友病因子的血浆浓缩物。经过病毒灭活方法处理的浓缩物应优于冷沉淀，因为它们同样有效且被认为更安全。
