The Relation between Serum Hepcidin, Ferritin, Hepcidin: Ferritin Ratio, Hydroxyurea and Splenectomy in Children with Î²-Thalassemia

Nagwa Abdallah Ismail; Sonia Adolf Habib; Ahmed A. Talaat; Naglaa Omar Mostafa; Eman A. Elghoroury

doi:10.3889/oamjms.2019.636

Authors

Nagwa Abdallah Ismail Pediatric Department, National Research Center, Cairo, Egypt
Sonia Adolf Habib Pediatric Department, National Research Center, Cairo, Egypt
Ahmed A. Talaat Pediatric Department, National Research Center, Cairo, Egypt
Naglaa Omar Mostafa Pediatric Hematology Department, Cairo University, Cairo, Egypt
Eman A. Elghoroury Clinical Pathology Department, National Research Center, Cairo, Egypt

DOI:

https://doi.org/10.3889/oamjms.2019.636

Keywords:

Hepcidin, Splenectomy, Ferritin, Thalassemia Intermedia, Thalassemia Major

Abstract

BACKGROUND: Hepcidin, a small peptide hormone, is established as the main regulator of iron homeostasis.

AIM: To estimate serum hepcidin, ferritin, and hepcidin: ferritin ratio in Î²-thalassemia patients and to determine the effect of splenectomy and hydroxyurea on serum hepcidin.

METHODS: A study was conducted on 30 thalassemia major (Î²TM), 29 thalassemia intermedia (Î²TI) and 29 healthy children's controls. Data were collected by patient interviewing where detailed history-taking and thorough clinical examinations were carried out. Serum ferritin and hepcidin were measured by ELISA assay (Bioneovan Co. Ltd Beijing, China).

RESULTS: Î’eta-thalassemia patients had higher serum ferritin, serum hepcidin and lower Hb and hepcidin: ferritin ratio compared to the controls (p < 0.001, 0.010, 0.001, 0.001) respectively. Î’-TM patients had higher mean serum hepcidin and serum ferritin compared to Î²-TI, with statistically significant difference (P = 0.042, P < 0.001, respectively). Twenty-one patients out of 29 Î²TI was on hydroxyurea therapy; these patients had significantly lower levels of serum ferritin (P < 0.004) and significantly higher levels of Hb (P < 0.004). Serum ferritin was statistically significantly higher in splenectomized patients P < 0.009. Serum hepcidin level was insignificantly higher in splenectomized patients than non-splenectomized patients (21.6 Â± 14.75, 17.76 Â± 10.01 ng/mL). Hepcidin showed a significantly positive correlation with hepcidin: ferritin ratio in all studied groups.

CONCLUSION: Serum hepcidin was elevated in Î²-thalassemia children with more evident elevation in Î²TM patients. Splenectomy played no major role in hepcidin regulation. Knowing that hepcidin in serum has a dynamic and multi-factorial regulation, individual evaluation of serum hepcidin and follow up, e.g. every 6 months could be valuable, and future therapeutic hepcidin agonists could be helpful in management of iron burden in such patient.