Hedner U, Kisiel W
J Clin Invest. 1983 Jun;71(6):1836-41. doi: 10.1172/jci110939.
Two patients with hemophilia A complicated with high-titer alloantibodies have been treated by repeated infusions of microgram quantities of pure human Factor VIIa. Patient 1 was presented with a gastrocnemius muscle bleeding that involved the knee joint. Upon treatment with Factor VIIa the circumference of the muscle decreased and joint mobility increased substantially. Patient 2 was given Factor VIIa concurrent with tranexamic acid in association with the extraction of two primary molars. No significant gingival bleeding occurred after Factor VIIa and tranexamic acid treatment. Furthermore, no deleterious side effects or increase of the alloantibody level were observed in either patient throughout the Factor VIIa infusion. These results, although limited and preliminary in nature, suggest that trace quantities of Factor VIIa can act as a Factor VIII bypassing activity and restore hemostasis in these patients.
两名患有血友病A并伴有高滴度同种抗体的患者，已通过反复输注微克量的纯人凝血因子VIIa进行治疗。患者1出现了累及膝关节的腓肠肌出血。经凝血因子VIIa治疗后，肌肉周长减小，关节活动度显著增加。患者2在拔除两颗乳磨牙时，凝血因子VIIa与氨甲环酸同时使用。凝血因子VIIa和氨甲环酸治疗后未发生明显的牙龈出血。此外，在整个凝血因子VIIa输注过程中，两名患者均未观察到有害的副作用或同种抗体水平升高。这些结果虽然在性质上有限且初步，但表明微量的凝血因子VIIa可以作为一种绕过凝血因子VIII的活性物质，恢复这些患者的止血功能。
