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Article Type

Original Study

Abstract

Objectives The current study was conducted to study the pathophysiology of iron overload in children with chronic hemolytic anemia (thalassemia major, thalassemia intermedia, and sickle cell anemia) and to assess the utility of soluble transferrin receptor (sTfR) for the evaluation of iron overload. Background Iron overload is a major complication of repeated blood transfusion in patients with chronic hemolytic anemia. sTfR, one of the main regulators of cellular iron homeostasis, is the truncated form of the tissue receptor. Patients and methods Sixty children with chronic hemolytic anemia (20 thalassemia major, 20 thalassemia intermedia, and 20 sickle cell anemia) were included, together with 20 age-matched and sex-matched controls. Clinical evaluation was performed for each child. Complete blood count along with serum ferritin and sTfR levels (using the ELISA technique) was assessed for both patients and controls. Results Both serum ferritin and sTfR levels were significantly higher in patients (2109±1350 ng/dl and 4.5±1.1 μg/ml, respectively) compared with controls and in patients with thalassemia major compared with those with thalassemia intermedia and those with sickle cell disease. Serum ferritin and sTfR levels were significantly correlated with age and with each other. Each of them negatively correlated with age at disease onset, time space between blood transfusions, and with hemoglobin level. Conclusion The sTfR level could contribute to and be used for the evaluation of iron overload in children with thalassemia and sickle cell disease.

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