Bowie E J, Solberg L A, Fass D N, Johnson C M, Knutson G J, Stewart M L, Zoecklein L J
J Clin Invest. 1986 Jul;78(1):26-30. doi: 10.1172/JCI112560.
Bone marrow from a normal male pig was transplanted into a related female pig with severe homozygous von Willebrand's disease (vWd). After engraftment the circulating leukocytes were of the male karyotype, and the platelets were strongly positive for von Willebrand factor (vWF) by indirect immunofluorescence. The average level of vWF was 1.96 U/dl and of ristocetin cofactor was 2.8 U/dl. The ear immersion bleeding time before transplantation was consistently more than 15 min and afterwards varied between 5 min and more than 15 min. Transfused vWF corrected the bleeding time at a level of 10 U/dl, which is lower than that required for a von Willebrand pig. We concluded that: the plasmatic compartment is only minimally replenished by the vWF from platelets and megakaryocytes; and the platelet vWF alone only partially corrects the abnormal tests of the hemostatic mechanism in severe vWd.
将一只正常雄性猪的骨髓移植到患有严重纯合性血管性血友病(vWd)的同系雌性猪体内。移植后,循环白细胞为雄性核型,通过间接免疫荧光法检测,血小板的血管性血友病因子(vWF)呈强阳性。vWF的平均水平为1.96 U/dl,瑞斯托霉素辅因子的平均水平为2.8 U/dl。移植前耳部浸入式出血时间持续超过15分钟,移植后在5分钟至超过15分钟之间变化。输入vWF在10 U/dl的水平时可纠正出血时间,这一水平低于血管性血友病猪所需的水平。我们得出以下结论:血浆部分仅由血小板和巨核细胞产生的vWF进行少量补充;仅血小板vWF只能部分纠正严重vWd中止血机制的异常检测结果。