Iron Overload and Chelation Therapy in Non-Transfusion Dependent Thalassemia

Int J Mol Sci. 2017 Dec 20;18(12):2778. doi: 10.3390/ijms18122778.

Abstract

Iron overload (IOL) due to increased intestinal iron absorption constitutes a major clinical problem in patients with non-transfusion-dependent thalassemia (NTDT), which is a cumulative process with advancing age. Current models for iron metabolism in patients with NTDT suggest that suppression of serum hepcidin leads to an increase in iron absorption and subsequent release of iron from the reticuloendothelial system, leading to depletion of macrophage iron, relatively low levels of serum ferritin, and liver iron loading. The consequences of IOL in patients with NTDT are multiple and multifactorial. Accurate and reliable methods of diagnosis and monitoring of body iron levels are essential, and the method of choice for measuring iron accumulation will depend on the patient's needs and on the available facilities. Iron chelation therapy (ICT) remains the backbone of NTDT management and is one of the most effective and practical ways of decreasing morbidity and mortality. The aim of this review is to describe the mechanism of IOL in NTDT, and the clinical complications that can develop as a result, in addition to the current and future therapeutic options available for the management of IOL in NTDT.

Keywords: iron chelation therapy; iron overload; liver iron concentration; non-transfusion dependent thalassemia; serum ferritin.

Publication types

  • Review

MeSH terms

  • Clinical Trials as Topic
  • Humans
  • Iron / metabolism
  • Iron Overload / diagnosis
  • Iron Overload / drug therapy*
  • Iron Overload / etiology
  • Siderophores / administration & dosage
  • Siderophores / adverse effects
  • Siderophores / pharmacology
  • Siderophores / therapeutic use*
  • Thalassemia / complications
  • Thalassemia / drug therapy*

Substances

  • Siderophores
  • Iron