Gralnick H R, Williams S B, McKeown L P, Rick M E, Maisonneuve P, Jenneau C, Sultan Y
Blood. 1986 Feb;67(2):465-8.
1-D-Amino(8-D-arginine)-vasopressin (DDAVP) infusion in three patients with type IIa von Willebrand's disease (vWD) resulted in a normalization of the factor VIII coagulant, factor VIII-related antigen, and von Willebrand factor (vWF) (ristocetin cofactor) activities and the bleeding time. The normalization of these hemostatic parameters persisted for four hours. Over the same time period there was a marked increase in the quantity of the vWF multimers when blood was collected in the presence of protease inhibitors. The vWF multimers present were even larger than the normal. When blood was collected in the absence of protease inhibitors, a smaller increase in the plasma vWF multimers was observed and fewer of the intermediate and larger vWF multimers were seen; multimers larger than those present in normal plasma were not visualized. The platelet vWF multimers and activities did not change with or without inhibitors. These studies suggest that there is a subgroup of patients with type IIa vWD who respond to DDAVP with complete normalization of their hemostatic abnormalities and whose vWF is sensitive to proteolysis.
对三名IIa型血管性血友病（vWD）患者输注1 - D - 氨基（8 - D - 精氨酸） - 血管加压素（DDAVP）后，凝血因子VIII、VIII相关抗原及血管性血友病因子（vWF）（瑞斯托霉素辅因子）活性和出血时间恢复正常。这些止血参数的正常化持续了4小时。在蛋白酶抑制剂存在的情况下采集血液时，同一时间段内vWF多聚体的量显著增加。所呈现的vWF多聚体甚至比正常的还要大。在不存在蛋白酶抑制剂的情况下采集血液时，血浆vWF多聚体的增加较小，且中间和较大的vWF多聚体较少见；未观察到比正常血浆中更大的多聚体。无论有无抑制剂，血小板vWF多聚体和活性均无变化。这些研究表明，存在一个IIa型vWD患者亚组，他们对DDAVP治疗反应良好，止血异常完全恢复正常，且其vWF对蛋白水解敏感。
