ISSN 2997-1036
International Journal of Hematology | Vol. 15, No. 5, May 2024 | pp. 33–40
DOI: 10.46882/2024/IJH/000171
Original Article
Title: Serum erythroferrone kinetics and absolute iron utilization markers in pediatric beta-thalassemia major variants
Names of Authors: A. I. Ibrahim¹, C. D. Balogun², E. O. Ojo³
Authors’ Affiliations: ¹Department of Haematology, Ahmadu Bello University, Zaria, Nigeria; ²Department of Paediatrics, University of Ilorin, Ilorin, Nigeria; ³Department of Chemical Pathology, Ladoke Akintola University of Technology, Ogbomoso, Nigeria
Abstract: Severe ineffective erythropoiesis drives profound hyperferritinemia and systemic iron loading in transfusion-dependent thalassemias, mediated by erythroblast-derived signaling peptides. This prospective clinical study evaluated serum erythroferrone (ERFE) kinetics, circulating hepcidin levels, and soluble transferrin receptor values in 45 pediatric patients presenting with confirmed beta-thalassemia major. Serum biomarker concentrations were quantified utilizing high-sensitivity enzyme-linked immunosorbent assays. Thalassemic children exhibited a five-fold increase in steady-state serum erythroferrone levels (mean 126.4 ± 24.5 pg/ml) compared to healthy controls (P < 0.001). This overproduction of erythroferrone correlated inversely with bioactive plasma hepcidin-25 levels (r = -0.58, P < 0.01), leading to unchecked intestinal iron absorption and toxic macrophage release despite significant tissue iron deposition (ferritin > 1200 ng/ml). Soluble transferrin receptor indices reflected immense marrow erythroid expansion. Tracking erythroferrone kinetics provides an accurate, non-invasive indicator of ineffective erythroid drive and systemic iron loading risk, helping clinicians optimize the timing for initializing iron chelation or therapeutic modulation before severe parenchymal target-organ damage manifests.
Keywords: Beta-thalassemia major, erythroferrone, hepcidin suppression, ineffective erythropoiesis, iron overload
Manuscript Timeline: Received: February 10, 2024; Revised: March 18, 2024; Accepted: April 05, 2024; Published: May 14, 2024