الفهرس | Only 14 pages are availabe for public view |
Abstract Hemophilia A is an X-linked inherited disease caused by mutations of FVIII gene which leads to qualitative or quantitative deficiency of coagulation FVIII that leads to bleeding. This is usually exaggerated with certain factors that lead to more occurrence of bleeding or severe forms as; plasma factor VIII level, presence of anti- FVIII inhibitors, age of the patient, activity, immune state, types and availability of treatment and socioeconomic state. In this study, we evaluate two antifibrinolytic factors (PAI-1 and TAFI) as contributors for increased bleeding tendency in hemophiliacs. The aim of this prospective case control study was to evaluate bleeding phenotype in patients with hemophilia A. As, a premature clot lysis is the more obvious explanation of delayed bleeding seen in hemophilics and variations of bleeding tendency between severe patients themselves and in some instances, between moderate and severe patients. Our study showed that there was no statistically significant correlation between bleeding score assessed by ISTH/BAT and patients severity. We investigated PAI-1 and TAFI levels as markers of fibrinolytic system. We found statistically highly significant difference between patients and controls as regard, PAI-1 and TAFI level (p˂0.001 in both) with higher level of TAFI in patients than controls and lower level of PAI-1in patients than controls. However, the two markers have no correlation with patients’ severity; they had slightly higher levels in severe patients than moderate ones. Our study showed significant correlation between PAI-1 level and bleeding phenotype, but there was no correlation between TAFI and bleeding phenotype. In conclusion, enhanced fibrinolytic system due to thrombin deficiency has an important role in bleeding tendency in hemophilia A regardless severity of the disease. |