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Abstract
Thrombotic events have been reported in adult thalassemic patients. To investigate this further, we measured hemostatic parameters in thalassemic children to identify possible predisposing factors in early childhood.
Objective: To assess hemostatic derangements in polytransfused children with beta-thalassemia major (beta-TM).
Methods: Complete blood count, prothrombin time, activated partial thromboplastin time, protein C, protein S, Antithrombin III (AT III), fibrinogen, D-dimer assay, serum iron, serum ferritin and liver function tests were measured in 54 patients and 30 controls using standard lab methods.
Results: Sixteen patients exhibited bleeding manifestations. None of the cases had thromboembolic phenomena. The average pretransfusion haemoglobin in the cases studied was 8.45 ± 1.6 g/dl, thrombocytopenia was seen in 33.3%, prolongation of prothrombin time was seen in 40.7% and prolongation of aPTT was seen in 46.3%. None of our patients had laboratory features of DIC. Protein C was low in 26.2%, protein S in 28.6% and AT III levels in 46.8% of cases. Mean fibrinogen levels and D-dimers were similar in cases and controls. Serum ferritin levels in the patients were high with a mean of 3709 ± 1625 ng/ml. Serum ferritin had a significant positive correlation with PT (r = 0.382) and ALT (r = 0.315) and a significant negative correlation with protein S (r = −0.376). Prolonged PT correlated with prolonged aPTT, low protein C, low protein S and serum ferritin levels. Protein C had a significant positive correlation with AT III. Low AT III activity correlated positively with age, aspartate transaminase and alanine transaminase. Average hemoglobin maintained correlated negatively with serum ferritin levels (r = −0.540), and AST (r = −0.417). Bleeding episodes correlated with age, liver size and number of transfusions.
Conclusion: Significant alterations in the hemostatic system already exist in polytransfused children with beta-thalassemia that make it a high-risk condition for both hemorrhagic manifestations and future development of thromboembolic events.