Erythropoiesis and Iron Parameters in Transfusion-dependent and Nontransfusion-dependent Thalassemias

J Pediatr Hematol Oncol. 2021 Jul 1;43(5):186-192. doi: 10.1097/MPH.0000000000002046.

Abstract

Introduction: To clarify mechanisms of ineffective erythropoiesis on iron metabolism, studies on erythroid factors that regulating hepcidin suppression have been carried out. The aim of the current study is to identify associations between erythropoiesis and iron homeostasis parameters in β-thalassemias.

Materials and methods: This study consisted of 83 subjects: 21 thalassemia major (TM), 20 thalassemia intermedia (TI), 20 thalassemia trait (TT), and 22 healthy subjects (HS). Erythroferrone (ERFE), hepcidin, growth differentiation factor-15 (GDF15), erythropoietin (EPO), and iron status parameters were measured.

Results: Our results showed that TM and TI patients had higher hepcidin than the TT and control groups. The hepcidin/ferritin in TM patients was significantly lower than the other groups. GDF15 in TM and TI patients was significantly higher than in the TT and control groups. Also, TI group had significantly higher ERFE concentration and EPO activity when compared with the TM, TT, and HS groups. EPO activity showed positive correlation with ERFE and GDF15 concentrations. We could not find any correlation between ERFE and hepcidin concentrations.

Conclusions: ERFE may be one of the parameters used to demonstrate erythropoietic activity level in thalassemias. More detailed studies are needed to clarify the role of ERFE in iron metabolism in the patients with thalassemias.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Blood Transfusion
  • Child
  • Erythropoiesis*
  • Female
  • Growth Differentiation Factor 15 / blood
  • Hepcidins / blood
  • Humans
  • Iron / blood*
  • Male
  • Peptide Hormones / blood
  • Thalassemia / blood*
  • Thalassemia / therapy*
  • Young Adult

Substances

  • Erfe protein, human
  • GDF15 protein, human
  • Growth Differentiation Factor 15
  • Hepcidins
  • Peptide Hormones
  • Iron