A review on the role of Hepicidin hormone in beta thalassemia major disease

Thalassemia is a haemoglobin disorder that is inherited. One or more globin chains of haemoglobin tetramers are produced less frequently or not at all, which causes uncontrollably high RBC destruction and severe anemia. An imbalance in globin chain synthesis causes damage to red blood cells in thalassemia. Hepcidin is severely reduced in thalassemia, and any increase in hepcidin function benefits erythropoiesis and iron metabolism. Clinical trials have been conducted on synthetic hepcidin and hepcidin mimetics. Nevertheless, their efficacy/safety profile has not been satisfactory. When taking hepcidin directly as a medication, it appears to be challenging to prevent iron over-restricted erythropoiesis. Indirect methods are numerous and still evolving, each with pros and cons. Targeting erythroferrone, the primary inhibitor of hepcidin expression, is the best strategy because iron-loading anaemias significantly raise its plasma concentrations. The development of pharmacological agonists and antagonists as well as hepcidin diagnostics should enhance the management of iron diseases.