血友病患者组织因子途径抑制物（TFPI）水平的研究

目的研究无临床出血及治疗干预的血友病患者组织因子途径抑制物（TFPI）水平与正常对照之间的差异,探讨TFPI在血友病出血机制中的意义。方法对65例血友病患者（血友病A 54例,血友病B 11例）及20例正常健康对照以ELISA法检测TFPI值,采用SPSS统计软件包进行统计学分析,说明二组的差异与差异的意义。结果血友病组与正常对照组TFPI均值分别为96.90±24.06 ng/ml及85.17±12.62 ng/ml,二组之间P〈0.05（P=0.006）,血友病患者TFPI水平明显高于正常对照;血友病A与血友病B组的TFPI均值分别为96.93±24.67 ng/ml及102.21±30.59 ng/ml,二组之间P〉0.05（P=0.537）。血友病A、B之间TFPI水平无差异。结论血友病患者TFPI水平明显高于正常对照。表明血友病出血机制除与因子Ⅷ、Ⅸ水平相关之外,尚与TFPI水平增高、凝血酶生成的负调节增强密切相关。提示抑制TFPI活性水平,有助于制止血友病出血。

Plasma Level of TFPI and its Significance in Patients with Hemophilia

Objective To explore the plasma level of tissue factor pathway inhibitor（TFPI） and its significance in patients with hemophilia.Methods Plasma level of TFPI was measured by ELISA in 65 patients with hemophilia（54 with hemophilia A,11 with hemophilia B）,which were not treated by factor replacement therapy during taking blood samples except 3 cases.Results Plasma level of TFPI in patients with hemophilia and on healthy controls were 96.90±24.06 ng/ml and 85.17±12.62 ng/ml respectively（P〈0.05）.Conclusion The results showed that the plasma level of TFPI in patients with hemophilia increased significantly and the bleeding of hemophilia was relative to the increasement of plasma level of TFPI.