Iron overload and chelation therapy in non-transfusion dependent thalassemia

Rayan Bou-Fakhredin, Abdul Hamid Bazarbachi, Bachar Chaya, Joseph Sleiman, Maria Domenica Cappellini, Ali T. Taher

Research output: Contribution to journalReview articlepeer-review

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.

Original languageEnglish
Article number2778
JournalInternational Journal of Molecular Sciences
Volume18
Issue number12
DOIs
Publication statusPublished - Dec 20 2017

Keywords

  • Iron chelation therapy
  • Iron overload
  • Liver iron concentration
  • Non-transfusion dependent thalassemia
  • Serum ferritin

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry

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