The Role of Erythropoiesis on Hepcidin Level in Polytransfused b-Thalassemia Major Patients

Abstract

AbstractBackground: Hepcidin peptide hormone is a main con-troller of iron homeostasis, itbecomes elevated in case of iron overload, however despite iron overload in b-Thalassemia major (b-TM) patients a contradictory decrease in Hepcidin occurs. The incompatible Hepcidin level is the main responsible factor causing the iron overload status in iron-loading anemias such as (b-TM) which contributes to organ dysfunctionand to iron toxicity.Aim of Study: To evaluate the conflicting effect of increased erythropoiesis in contrast to the effect of iron overloading on Hepcidin serum level and its correlation with iron status in (b-TM) patients.Subjects and Methods: For all patients and controls Complete Blood Count (CBC), serum assaysof Hepcidin, iron, ferritin, transferrin, HCVAbs, HBs Ag, CRP and serum level were performed.Results: Hepcidin level was significantly lowered in (b-TM) patients compared to controls with high significant difference (p=0.00). There was no correlation between serum Hepcidin and ferritin level, neither was between Hepcidin and serum iron, transferrin, Hb level and reticulocyte count in the study group. Hepcidin/Ferritin ratio showed high significance difference (p<0.000) with mean value in the study group 0.056 vs. 4.75 in controls pointing to an incom-patible levels of Hepcidin to iron overload status. Hepcidin level wasnegative correlatedwith age (p<0.05).Conclusion: Hepcidin was markedly decreased in the study group with no significant correlation between serum Hepcidin and ferritin level as a marker of iron overload in thalassemia major. Hepcidin deficiency is the main contributing factor of iron overload in b thalassemia which results from a strong suppressive effect of the high erythropoietic activity on Hepcidin expression. Hepcidin-ferritin ratio was markedly depressed <1 in all b thalassemia major patients which indicating suppression of Hepcidin out of proportion with the degree of iron loading.