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目的探讨遗传性凝血因子V(FV)、X(FX)和XI(FXI)缺陷及FV和凝血因子VⅢ(FVⅢ)联合缺陷(F5F8D)患者的血浆凝血因子活性、凝血酶生成曲线各参数和临床出血症状之间的关系。方法采集遗传性FV(n=24)、FX(n=14)和FXI(n=18)缺陷及F5F8D(n=8)患者及携带者的外周血进行常规出凝血检查及自动校正凝血酶曲线法凝血酶生成试验。结果凝血酶生成曲线的各项参数与FV促凝活性(FV:C)及FX促凝活性(FX:C)存在双曲线趋势关系。FV或FX缺陷患者血浆中缺陷因子的活性只要达到正常人的3%,凝血酶生成潜力和峰值就达到正常值的一半以上。FV:C≤6.9%或FX:C≤2.2%的患者具有出血症状,活化部分凝血活酶时间(APTT)、凝血酶原时间(PT)、延迟时间及达峰时间都有不同程度的延长。具有重度出血症状的3例患者的缺陷因子(FV或FX)活性均≤1.5%,APTT、PT、延迟时间和达峰时间都明显延长,且ETP=0。结论凝血酶生成试验的各项参数与凝血因子FV、FX活性及出血症状的发生及严重程度具有相关性,凝血酶生成试验结合APTT、PT及凝血因子活性可以作为评估遗传性凝血因子缺陷症患者出血倾向的有效手段。
Objective To investigate the changes of plasma coagulation factor (FV), X (FX) and XI (FXI) deficiencies and FV and FVIII combined defects (F5F8D) The relationship between bleeding symptoms. Methods Peripheral blood samples of patients with FV (n = 24), FX (n = 14), FXI (n = 18) and F5F8D (n = 8) and carriers were routinely checked for coagulopathy and automatically calibrated thrombin France thrombin generation test. Results The parameters of thrombin generation curve showed a hyperbolic trend with respect to FV procoagulant activity (FV: C) and FX procoagulant activity (FX: C). Defective FV or FX deficient plasma plasma factor as long as the activity of 3% of normal, thrombin generation potential and peak value reached more than half of normal. Patients with FV: C ≤ 6.9% or FX: C ≤ 2.2% had hemorrhagic symptoms, prolonged APTT, PT, delay and peak time. The three patients with severe hemorrhagic symptoms had a FV or FX deficiency activity of ≤1.5%. The APTT, PT, delay time and peak time were significantly prolonged, and ETP = 0. Conclusion The parameters of thrombin generation test are related to the occurrence and severity of coagulation factors FV and FX and bleeding symptoms. Thrombin generation test combined with APTT, PT and clotting factor activity can be used as a method to evaluate patients with hereditary coagulation factor deficiency Effective means of bleeding tendency.