Ghosh Kanjaksha, Mota Leenam, Shetty Shrimati, Kulkarni Bipin
National Institute of Immunohaematology (ICMR), KEM Hospital, Parel, Mumbai, India.
Blood Coagul Fibrinolysis. 2008 Sep;19(6):577-80. doi: 10.1097/MBC.0b013e328307089d.
The modern thrombin generation tests describe different phases of generation of thrombin that is initiation, amplification and inhibition of thrombin generation as well as the integral amount of generated thrombin. We investigated 55 patients with congenital deficiencies of different coagulation factors and analysed the relationship between the nature and the concentration of clotting factors, with different parameters of thrombin generation curve that is lag time, peak, time to peak and the area under curve or endogenous thrombin potential. The endogenous thrombin potential was unaffected by severe deficiency of factors XI and XII, and reduced in factor IX, VII and factor V and VIII deficiencies. The lag time was significantly prolonged in cases of severe factor VII, X and V deficiencies, and was almost normal in cases of factors VIII, IX, combined factors V and VIII, factor XI, XII and XIII deficiencies. The peak height was severely affected in cases of severe factor X, V, VIII and IX deficiency and combined deficiency of multiple vitamin K dependant coagulation factors, and significantly reduced in factor VIII, V, X, XIII and combined vitamin K deficiency.In all the patients with less than 40% thrombin generation, the clinical symptoms were severe. Bleeding symptoms were restricted to epistaxis and ecchymosis when thrombin generation was more than 90% of the normal. In the cases of combined deficiency of factors V and VIII all the values were intermediate as they exhibit mild deficiencies of both factors V and VIII and correlated well with the clinical symptoms. Endogenous thrombin potential of inherited isolated deficiencies of coagulation factors may thus provide an interesting insight about involvement of the deficient factor(s) at different phases of thrombin generation.
现代凝血酶生成试验描述了凝血酶生成的不同阶段，即凝血酶生成的起始、放大和抑制以及生成的凝血酶总量。我们研究了55例不同凝血因子先天性缺乏的患者，并分析了凝血因子的性质和浓度与凝血酶生成曲线的不同参数（即滞后时间、峰值、达到峰值的时间以及曲线下面积或内源性凝血酶潜力）之间的关系。内源性凝血酶潜力不受因子XI和XII严重缺乏的影响，而在因子IX、VII以及因子V和VIII缺乏时降低。在严重因子VII、X和V缺乏的情况下，滞后时间显著延长，而在因子VIII、IX、联合因子V和VIII、因子XI、XII和XIII缺乏的情况下，滞后时间几乎正常。在严重因子X、V、VIII和IX缺乏以及多种维生素K依赖凝血因子联合缺乏的情况下，峰值高度受到严重影响，而在因子VIII、V、X、XIII和联合维生素K缺乏时显著降低。在所有凝血酶生成低于40%的患者中，临床症状严重。当凝血酶生成超过正常的90%时，出血症状仅限于鼻出血和瘀斑。在因子V和VIII联合缺乏的情况下，所有值均处于中间水平，因为它们表现出因子V和VIII的轻度缺乏，且与临床症状密切相关。因此，遗传性孤立性凝血因子缺乏的内源性凝血酶潜力可能为缺乏因子在凝血酶生成不同阶段的参与情况提供有趣的见解。
