凝血酶生成试验在血友病患者中的应用

目的：探讨凝血酶生成试验在血友病患者中的应用。方法：收集134例血友病患者[其中血友病A（HA）114例，血友病B（HB）20例1的临床资料，根据FⅧ：C／FIX：C不同，将无FⅧ／FⅨ抑制物的132例HA／HB患者分成重型（FⅧ：C／FIX：C〈1％）、中型（FVIU：C／FIX：C1％～5％）及轻型（FⅧ：C／FIX：C6％～25％）3组，检测凝血酶生成、FⅧ：C／FIX：C及其抑制物。结果：重型HA／HB患者与轻型者比较，出血频度及凝血酶生成各指标差异均有统计学意义（P〈0．01）；而重型者与中型者比较，除延迟时间和达峰时间差异有统计学意义外（P〈0．01），其余指标差异均无统计学意义；中型者与轻型者比较，除延迟时间和达峰时间差异无统计学意义外，其余指标差异均有统计学意义（P〈0．01）。HA／HB患者的出血频度与FⅧ：C或FIX：C无关（P=0．16），而与凝血酶生成潜力（ETP）密切相关（P=0．0001）：结论：凝血酶生成试验可作为预测HA／HB患者出血风险的有效手段。

Use of thrombin generation test in hemophilia patients

Objective To explore the significance of thrombin generation test in patients with hemophilia and its relation with hemorrhage. Methods The clinical data of 134 hemophilia patients （114 hemophilia A patients, 20 hemophilia B patients） were collected. One hundred and thirty two hemophilia A and B patients without inhibitor were divided into severe type （FⅧ：C/FⅨ：C 〈1%）, moderate type （FⅧ：C/FⅨ：C 1%-5%） and mild type （FⅧ：C/FⅨ：C 6%-25%） in accordance with the level of FⅧ：C/FⅨ：C. Thrombin generation, FⅧ：C/FⅨ：C activity and inhibitors were tested. Results Significant differences in bleeding frequency and parameters of thrombin generation test were seen between severe type and mild type hemophilia patients （P〈0.O1）. Except significant difference seen in lag time （LT） and time to peak （ttPeak） （P〈0.01）, no significant difference was found in other parameters between severe type and moderate type hemophilia patients. Whereas significant difference was found in all other parameters （P〈0.01） except LT and ttPeak between moderate and mild type hemophilia patients. Bleeding frequency had no correlation with FⅧ：C/FⅨ：C activity（P= 0.16）, but was closely correlated with endogenous thrombin potential （ETP） （P=0.0001）. Conclusions Thrombin generation test can be used as a tool for predicting the risk of bleeding of hemophilia patients.