Iron overload: The achilles heel of β-thalassemia

Transfus Clin Biol. 2024 Aug;31(3):167-173. doi: 10.1016/j.tracli.2024.06.001. Epub 2024 Jun 6.

Abstract

Systematic transfusions coupled with iron chelation therapy have substantially improved the life expectancy of thalassemia patients in developed nations. As the human organism does not have a protective mechanism to remove excess iron, iron overload is a significant concern in thalassemia, leading to organ damage, especially in the heart and liver. Thus, iron chelation therapy is crucial to prevent or reverse organ iron overload. There are three widely used iron chelators, either as monotherapy or in combination. The choice of iron chelator depends on several factors, including local guidelines, drug availability, and the individual clinical scenario. Despite treatment advancements, challenges persist, especially in resource-limited settings, highlighting the need for improved global healthcare access. This review discusses clinical management, current treatments, and future directions for thalassemia, focusing on iron overload and its complications. Furthermore, it underscores the progress in transforming thalassemia into a manageable chronic condition and the potential of novel therapies to further enhance patient outcomes.

Keywords: Blood transfusion therapy; Hemoglobinopathies; Iron chelation therapy; Iron overload; β-thalassemia.

Publication types

  • Review

MeSH terms

  • Blood Transfusion
  • Chelation Therapy
  • Humans
  • Iron / metabolism
  • Iron Chelating Agents* / therapeutic use
  • Iron Overload* / etiology
  • beta-Thalassemia* / complications
  • beta-Thalassemia* / therapy

Substances

  • Iron Chelating Agents
  • Iron