Menache D, Aronson D L
Biomedical Services, Amarican Red Cross, Washington, DC, USA.
Thromb Haemost. 1997 Jul;78(1):566-70.
During the past 10 years the quality of plasma derived virus inactivated products containing von Willebrand factor (vWF) has improved and the ratios of ristocetin cofactor activity (vWF:RCo) to von Willebrand factor antigen (vWF:Ag) have increased. There are, however, considerable variations in AHF-vWF products with ranges from 0.25 to 1.4 unit of vWF:RCo for each unit of vWF:Ag, and 0.5 to 5.3 units of vWF:RCo for each factor VIIIC (FVIIIC) unit. The availability of a vWF product depleted of FVIII has allowed pharmacokinetic studies of the vWF protein. There have been no dose response studies, dosage regimens remain empirical and are still, except in rare instances, based on FVIIIC dosage. Current concentrates are as effective as cryoprecipitate in the management of patients with vWD non responsive to DDAVP. These concentrates should be preferred to cryoprecipitate which carries a risk of virus transmission.
在过去10年中，含血管性血友病因子（vWF）的血浆源性病毒灭活产品质量有所提高，瑞斯托霉素辅因子活性（vWF:RCo）与血管性血友病因子抗原（vWF:Ag）的比率有所增加。然而，抗血友病因子 - vWF产品存在相当大的差异，每单位vWF:Ag的vWF:RCo范围为0.25至1.4单位，每单位凝血因子VIIIC（FVIIIC）的vWF:RCo范围为0.5至5.3单位。不含FVIII的vWF产品的可得性使得能够对vWF蛋白进行药代动力学研究。目前尚无剂量反应研究，给药方案仍基于经验，并且除了极少数情况外，仍然基于FVIIIC剂量。在治疗对去氨加压素无反应的血管性血友病（vWD）患者时，目前的浓缩物与冷沉淀一样有效。与存在病毒传播风险的冷沉淀相比，这些浓缩物应更受青睐。
