Endothelial Activation Markers in Polytransfused Children with Beta Thalassemia: Study from a Tertiary Care Centre in
Tushar Subhash Pallewar1, Kusha Sharma1, Sunita Sharma2
1Lady Hardinge Medical College and Associated Hospitals, New Delhi, 110055 India.
Insights
Children with beta thalassemia major show increased markers of endothelial activation and coagulation, indicating a subclinical hypercoagulable state. These findings suggest endothelial activation markers could predict thrombotic risks in these patients.
Area of Science:
- Hematology
- Vascular Biology
- Pediatric Medicine
Background:
- Beta thalassemia major is linked to a prothrombotic state.
- Endothelial dysfunction and hemostatic alterations are implicated.
- Soluble Intercellular adhesion molecule (sICAM-1) and E-selectin are key markers.
Purpose of the Study:
- To investigate serum levels of sICAM-1 and E-selectin in children with beta thalassemia major.
- To assess the association of these markers with serum ferritin and D-dimer levels.
Main Methods:
- Study included 62 children with beta thalassemia major and 26 healthy controls.
- Coagulation tests (PT, APTT, D-dimer) and endothelial markers (ICAM-1, E-selectin) were analyzed.
- Serum ferritin levels were also measured.
Main Results:
- Patients exhibited significantly higher PT, APTT, and D-dimer levels compared to controls.
- Mean ICAM-1 and E-selectin levels were significantly elevated in beta thalassemia major patients.
- No significant correlation was found between endothelial markers and ferritin, PT, APTT, or D-dimer.
Conclusions:
- Subclinical activation of coagulation and fibrinolysis is present in polytransfused children with beta thalassemia major.
- Endothelial activation markers may serve as early indicators of endothelial dysfunction.
- These markers could aid in assessing thrombotic complication risks in beta thalassemia.
Abstract:
Beta thalassemia major is associated with a subclinical hypercoagulable state. Endothelial activation markers like soluble Intercellular adhesion molecule (sICAM-1) and E-selectin have been implicated in the pathogenesis of endothelial dysfunction and hemostatic alterations. In this study we aimed to study serum levels of sICAM-1 and E-selectin in polytransfused children with β thalassemia major and their association with serum ferritin and D-dimer levels. Sixty-two polytransfused β-thalassemia major children aged between 5 and 17 years and 26 age and gender matched healthy controls were enrolled in the study. Complete blood count with peripheral smear, liver function tests, serum ferritin, coagulation tests [PT, APTT, D-dimer] and endothelial activation marker tests [ICAM-1 and E-selectin] were performed. PT, APTT and D-dimer levels were significantly higher in beta-thalassemia major patients than in control group (p = 0.003, p < 0.001, p < 0.001 respectively). Mean ICAM-1 and E-selectin levels were 731.34 ± 343.97 ng/ml and 111.75 ± 40.13 ng/ml respectively which were significantly higher than control group (p < 0.001, p < 0.001 respectively). No significant correlation of ICAM-1 and E-selectin was observed with serum ferritin, PT, APTT and D-dimer levels. The findings of the present study suggest that there is ongoing subclinical activation of coagulation cascade and fibrinolytic system in these patients. Endothelial activation markers may be used as early indicators of endothelial dysfunction to assess the thrombotic complications in beta thalassemia.
