Pathophysiology of Beta-Thalassaemia: (Part 3) [Iron Overload]

Ne Win1

 

Published in Volume 67, No. 4, 2025 October – December issue

https://doi.org/10.64455/xmma0013

 

Based on the requirement for blood transfusion therapy, beta-thalassaemia (β-thal) can be classified as transfusion-dependent β-thal (TDT) and non-transfusion-dependent β-thal (NTDT). There are three categories of β-thal based on how severe the disorder is; the β-thal major, β-thal intermedia, and the β-thal carrier. Iron overload develops from increased intestinal iron absorption signaled by ineffective erythropoiesis, while it can also be secondary to regular transfusions, which have been conventionally used to manage severe forms of the disease.

 

Iron is an essential trace element, but it is also toxic in excess. Therefore iron homeostasis is a critical process due to the dangers caused by the body’s large amounts of free reactive iron. Iron metabolism is mainly regulated by substances secreted by the liver such as Transferrin (TF), Transferrin receptor (TFR), Ferritin and Hepcidin. They are vital in maintaining systemic iron equilibrium.

 

  1. Senior Consultant Pathologist
    Research and Development Sub-Committee, Myanmar Academy of Medical Science (MAMS)

Corresponding author: [email protected]

https://orcid.org/0009-0001-7253-0022