Hepcidin and iron regulation in chronic hemolytic anemia

Abstract

Objectives The aim of the study was to investigate the changes in serum hepcidin and ferritin level in chronic hemolytic anemia (β-thalassemia major and sickle cell disease) to clarify the relation between hepcidin level and iron regulation. Background The hallmark of hemolytic anemia is reduced life span of RBCs. In β-thalassemia there is excess α-globin chain relative to β-globin chain; α-globin tetramers are formed, and interact with RBC membranes and shorten RBC survival, leading to anemia and increased erythrocyte production. Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S, which distorts the shape of RBCs, especially with hypoxia. Frequent blood transfusion without proper iron chelation causes hemochromatosis. Recently, the predominant negative regulator of iron absorption and iron release was discovered - namely, hepcidin, the 25-amino acid peptide produced by hepatocytes. Patients and methods The study was carried out on 55 children, categorized into 23 children with thalassemia major, 12 with sickle cell disease, and 20 apparently healthy control children, who were matched in age, sex, and socioeconomic standard. Complete blood count, analysis of serum ferritin, hemoglobin electrophoresis, liver and renal function tests, and evaluation of serum hepcidin level using enzyme-linked immunosorbent assay kits were performed. Results Ferritin level in thalassemia and sickle cell patients was significantly higher than in controls ( P P P P = 0.812). Conclusion In future, hepcidin level may allow more accurate assessment of the degree of iron overload and iron misdistribution.