Basu, S.; Rahaman, M.; Dolai, T.K.; Shukla, P.C.; Chakravorty, N. Understanding the Intricacies of Iron Overload Associated with β-Thalassemia: A Comprehensive Review. Thalass. Rep.2023, 13, 179-194.
Basu, S.; Rahaman, M.; Dolai, T.K.; Shukla, P.C.; Chakravorty, N. Understanding the Intricacies of Iron Overload Associated with β-Thalassemia: A Comprehensive Review. Thalass. Rep. 2023, 13, 179-194.
Basu, S.; Rahaman, M.; Dolai, T.K.; Shukla, P.C.; Chakravorty, N. Understanding the Intricacies of Iron Overload Associated with β-Thalassemia: A Comprehensive Review. Thalass. Rep.2023, 13, 179-194.
Basu, S.; Rahaman, M.; Dolai, T.K.; Shukla, P.C.; Chakravorty, N. Understanding the Intricacies of Iron Overload Associated with β-Thalassemia: A Comprehensive Review. Thalass. Rep. 2023, 13, 179-194.
Abstract
β-thalassemia, a congenital genetic hematological disorder characterized by decreased or absence of β-globin chains, leads to decrease in levels of Hemoglobin A. The affected individuals can be categorized into two cohorts based on transfusion dependency: transfusion dependent thalassemia (TDT) and non-transfusion dependent thalassemia (NTDT). Remarkably, despite the primary pathology lying in β-globin chain depletion, β-thalassemia exhibits an intriguing association with iron overload. Iron metabolism, a tightly regulated physiological process, reveals a complex interplay in these patients. Over time, both cohorts of β-thalassemic individuals develop iron overload, albeit through distinct mechanisms.
Addressing the diverse complications arising due to iron overload in β-thalassemic patients, the utilization of iron chelators has gained a lot of significance. With varying efficacies, routes of administration, and modes of action, different iron chelators offer unique benefits to patients. In the Indian context, three commercialized iron chelators have emerged, showcasing a high adherence rate to the iron chelator-based treatment regimens among β-thalassemic individuals. In this review, we explore the intriguing connection between β-thalassemia and iron overload, shedding light on the intricate mechanisms at play. We delve into the intricacies of iron metabolism, unveiling the distinct pathways leading to iron accumulation in these patients. Additionally, we critically evaluate the therapeutic efficacy of different iron chelators, emphasizing their respective advantages in managing iron overload complications. Through this comprehensive analysis, we aim to deepen our understanding of β-thalassemia and iron overload, paving the way for optimized treatment strategies. Ultimately, our findings provide valuable insights into improving the care and outcomes of individuals affected by β-thalassemia.
Keywords
β-thalassemia; iron overload; iron chelators; TDT; NTDT
Subject
Medicine and Pharmacology, Hematology
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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