Abstract Number: PB0604
Meeting: ISTH 2021 Congress
Background: Patients with hemophilia A display varied bleeding phenotypes not correlated with degree of deficiency of factor VIII level.previous studies also releaved that premature clot lysis occur in plasma deficient in components of intrinsic pathway.So, bleeding in Hemophilia may be due to enhanced fibrinolysis n addition to factor VIII deficiency.
Aims: We aimed to find correlation between bleeding variations in hemophilic patients and their level of Plasminogen Activatable Fibrinolysis Inhibitor 1(PAI-1) and Thrombin Activatable Fibrinolysis Inhibitor (TAFI) levels as antifibrinolytic factors.
Methods: Case control study was conducted on 26 malechildren patients diagnosed with Hemophilia A and 14 apparently healthy male children subjects with matched age as a control group.
The International Society of Thrombosis Bleeding Assessment Tool (ISTH/BAT) was used to assess bleeding score in patients. Patients are divided according to their bleeding score into intense and non-intense haemorrhagic groups. Plasminogen Activator Fibrinolysis Inhibitor (PAI1) and Thrombin Activatable Fibrinolysis Inhibitor (TAFI) levels were measured by enzyme-linked immunosorbent assay (ELIZA).
Results: As compared to controls, hemophilic patients had significantly high bleeding score, low PAI 1 level and high TAFI level. There was no significant correlation between bleeding score by ISTH/BAT and patient severity. PAI 1 and TAFI level have no significant correlation with patient severity. We found significant correlation between PAI-1 level and bleeding intensity,however we couldn’t able to detect such correlation between TAFI level and bleeding intensity.
Conclusions: We concluded that there are significant correlation between patients and control as regard PAI-1 and TAFI levels. That means presence of enhanced fibrinolysis in hemophilic.PAI-1 significantly correlated with bleeding intensety,while TAFI didn’t correlate with it.
To cite this abstract in AMA style:Elhefnawy S, Abd Elrehim M, ME. Can Plasminogen Activator Inhibitor 1 and Thrombin Activitable Fibrinolysis Inhibitor Explain Bleeding Phenotype in Hemophilia A? [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/can-plasminogen-activator-inhibitor-1-and-thrombin-activitable-fibrinolysis-inhibitor-explain-bleeding-phenotype-in-hemophilia-a/. Accessed December 6, 2023.
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