الفهرس 
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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.