Assessment of plasminogen activator inhibitor-1(PAI1) and thrombin activitable fibrinolysis inhibitor (TAFI) in Egyptian children with hemophilia A

Abstract

Patients with hemophilia A display varied bleeding phenotypes not correlated with degree of deficiency of factor VIII level. We investigated Plasminogen Activator Inhibitor 1(PAI1) level and Thrombin Activatable Fibrinolysis Inhibitor (TAFI) also known as Carboxypeptidase B2 (CPB2) level in Patients with hemophilia A and their possible correlation with bleeding tendency. Twenty-six patients attending in hematology unit of pediatric department were included in this study. In addition, fourteen apparently healthy subjects matched ages and genders were included as control group. The International Society of Thrombosis Bleeding Assessment Tool (ISTH/BAT) was used to assess bleeding score in patients. Plasma levels of Plasminogen Activator Fibrinolysis Inhibitor (PAI1) and Thrombin Activatable Fibrinolysis Inhibitor (TAFI) zymogen were measured by enzyme-linked immunosorbent assay (ELIZA). 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. PAI 1 level was statistically significant different between intense and non-intense hemorrhagic groups, while TAFI level has no significant correlation with bleeding phenotype. PAI 1 and TAFI levels had significantly correlation between patients and controls. PAI-1 level had statistically significant correlation with bleeding phenotype, while TAFI level failed to show any correlation between intense and non-intense hemorrhagic groups. So, PAI-1 levels may have predictive value of bleeding tendency in hemophiliacs.

Keywords:

• CarboxypeptidaseB2
• factor VIII
• international society of thrombosis bleeding assessment tool
• plasminogen activator fibrinolysis inhibitor 1
• thrombin activatable fibrinolysis inhibitor

Authors’ contributions

All authors meet the ICMJE authorship criteria, in that all authors made substantial contributions to the conception and design of the study, acquisition of data, or analysis and interpretation of data. In additional, all authors were involved in drafting the manuscript or revising it critically for important intellectual con-tent, and all provided final approval of the version to be submitted.

Compliance with ethical standards

Conflict of invterest

The authors declare that they have no conflict of interest

Ethics approval

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008.Consent to participate Informed consent was obtained from all patients for being included in the study.

Consent for publication

All authors approved the manuscript and gave their consent for publication.

Availability of data and material

The datasets generated and analyzed during the study are available from the corresponding author on reason-able request.

Code availability

Not applicable.

Additional information

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.