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Thomas Siegemund, Sirak Petros, Annelie Siegemund, Ute Scholz, Lothar Engelmann
Thrombin generation in severe haemophilia A and B: the endogenous thrombin potential in platelet-rich plasma
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 200 109/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 100 109/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/109 platelets/l and in haemophilia B by 0.319 0.085 FU/109 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.
Thrombosis and Haemostasis, Schattauer
Print ISSN: 0340-6245
Volume: 90, 11/2003
Pages: 781 - 786