Siegemund Thomas, Petros Sirak, Siegemund Annelie, Scholz Ute, Engelmann Lothar
Clinical Haemostaseology, Medical Clinic I, University of Leipzig, Leipzig, Germany.
Thromb Haemost. 2003 Nov;90(5):781-6. doi: 10.1160/TH03-01-0027.
Thrombin generation was investigated in platelet-rich plasma (PRP) from 11 healthy controls, 17 patients with severe haemophilia A and 7 patients with severe haemophilia B. Mean endogenous thrombin potential (ETP) in arbitrary fluorescence units (FU) was 226.9 +/- 44.6, 186.4 +/- 22.5, 154.2 +/- 41.3 in controls, haemophilia A and B, respectively, all at a platelet count of 200 x 10(9)/l (p = 0.004 for controls vs. haemophilia A, p = 0.003 for controls vs. haemophilia B, no significant difference between haemophilia A and B). The contribution of FVIII to thrombin generation in haemophilia A was 1.31 +/- 0.16 FU/% of FVIII:C activity, while for FIX in haemophilia B this was 0.80 +/- 0.21 FU/% of FIX activity. There was an almost linear relationship between increasing platelet count and thrombin generation up to a mean platelet count of 100 x 10(9)/l. Further increase in platelet count has only a marginal influence on thrombin generation. Platelets increase ETP in haemophilia A by 0.184 +/- 0.022 FU/10(9) platelets/l and in haemophilia B by 0.319 +/- 0.085 FU/10(9) platelets/l, and this was significantly different between the two groups (p = 0.0002). This influence of plate-lets diminishes with increasing concentration of either FVIII or FIX. In conclusion, there is a difference in thrombin generation between haemophilia A and B, and this may be attributed to the role of platelets in the assembly of the tenase complex on their surface.
对11名健康对照者、17名重度甲型血友病患者和7名重度乙型血友病患者的富血小板血浆（PRP）中的凝血酶生成情况进行了研究。以任意荧光单位（FU）表示的平均内源性凝血酶潜力（ETP）在对照者、甲型血友病患者和乙型血友病患者中分别为226.9±44.6、186.4±22.5、154.2±41.3，所有样本的血小板计数均为200×10⁹/L（对照者与甲型血友病患者相比，p = 0.004；对照者与乙型血友病患者相比，p = 0.003；甲型血友病患者与乙型血友病患者之间无显著差异）。在甲型血友病中，FVIII对凝血酶生成的贡献为1.31±0.16 FU/ FVIII:C活性的百分比，而在乙型血友病中，FIX的这一贡献为0.80±0.21 FU/ FIX活性的百分比。在平均血小板计数达到100×10⁹/L之前，血小板计数增加与凝血酶生成之间几乎呈线性关系。血小板计数进一步增加对凝血酶生成的影响很小。血小板使甲型血友病患者的ETP增加0.184±0.022 FU/10⁹血小板/L，使乙型血友病患者的ETP增加0.319±0.085 FU/10⁹血小板/L，两组之间存在显著差异（p = 0.0002）。随着FVIII或FIX浓度的增加，血小板的这种影响会减弱。总之，甲型血友病和乙型血友病在凝血酶生成方面存在差异，这可能归因于血小板在其表面组装凝血酶原酶复合物中的作用。
