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.
Keywords: Beta thalassemia; D-dimer; E-selectin; ICAM-1.
© Indian Society of Hematology and Blood Transfusion 2021.