Hepcidin/Ferritin Ratio in Patients with Beta Thalassemia. A Single-Center Experience

Abstract

Abstract Background Hepcidin, a main regulator of iron hemostasis, can potentially be used as a marker for iron overload in patients with thalassemia; hence can be used to evaluate the efficacy of chelation therapy. Aim We hypothesize that serum hepcidin and hepcidin/ferritin ratio might be better markers for iron load and iron load distribution characteristics in different thalassemia syndromes. Subsequently we aimed to estimate serum hepcidin, ferritin, and hepcidin: ferritin ratio in patients with beta-thalassemia syndromes. Patients and Methods A prospective study was conducted on 42 patients with beta- thalassemia major and 40 patients with beta-thalassemia intermedia compared to 20 age and gender-matched healthy controls. For all patients, serum ferritin, and hepcidin were measured by ELISA assay then the hepcidin /ferritin ratio was calculated. Results All patients (100%) with beta-thalassemia major and 26 patients (65%) with beta- thalassemia intermedia were transfusion dependent. Sixty-one patients (74%) were on regular iron chelators, 49 of them (80%) were on desafirox with a median dose of 20 mg/kg, 40 patients (100%) of beta thalassemia intermedia were on hydroxyurea. Patients with thalassemia syndromes had higher mean serum ferritin (3000 ng/ml), median serum hepcidin of 162 ng/ml, lower mean hemoglobin (8.2 gm/dl) and hepcidin: ferritin ratio of 0.15 compared to control (P < 0.001) who had median serum ferritin (73 ng/ml), median serum hepcidin of (62.8 ng/ml), mean hemoglobin (11.03 gm/dl) and hepcidin: ferritin ratio of 0.71. Beta thalassemia major patients had a higher median serum hepcidin level of 256 ng/ml, and a lower hepcidin /ferritin ratio of 0.18 compared to patients with beta-thalassemia intermedia (p < 0.001, p < 0.001 respectively). Hepcidin level was negatively correlated with the age of onset, frequency of transfusion. Conclusion Serum hepcidin level was elevated in beta-thalassemia children with more evident elevation in beta-thalassemia major patients. Also hepcidin: ferritin ratio was elevated in normal controls and low in thalassemia intermedia differentiating both entities. The younger the age of onset of Thalassemia, the higher the need for transfusion, and the dose of desafirox, the higher was the hepcidin level.