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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]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(FⅧ:C/FⅨ:C 1%～5%)及轻型(FⅧ:C/FⅨ:C 6%～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。
Objective: To investigate the application of thrombin generation test in patients with hemophilia. Methods: The clinical data of 134 patients with hemophilia [including 114 cases of haemophilia A (HA) and 20 cases of hemophilia B (HB)] were collected. According to F Ⅷ: C / F Ⅸ: C, The 132 patients with HA / HB inhibitors were divided into severe (F Ⅷ: C / F Ⅸ: C 1%), medium (F Ⅷ: C / F Ⅸ: % ~ 25%) 3 groups were detected thrombin generation, F Ⅷ: C / F Ⅸ: C and its inhibitors. Results: The frequency of haemorrhage and the indexes of thrombin generation in severe HA / HB patients were significantly different from those in mild type (P <0.01), while those in heavy type and medium type were higher than those in mild type Statistical significance (P <0.01), the remaining indicators were no significant difference between the medium and light type, except for the delay time and peak time was not statistically significant, the other indicators were statistically significant differences (P <0.01). The frequency of bleeding in patients with HA / HB was not related to FⅧ: C or FⅨ: C (P = 0.16), but to thrombin generation potential (ETP) (P = 0.0001). Conclusion: The thrombin generation test can be used as an effective method to predict the risk of bleeding in patients with HA / HB.