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Int. J. Mol. Sci. 2017, 18(12), 2778; https://doi.org/10.3390/ijms18122778

Iron Overload and Chelation Therapy in Non-Transfusion Dependent Thalassemia

1
Department of Internal Medicine, American University of Beirut Medical Center, Beirut 11-0236, Lebanon
2
Faculty of Medicine, American University of Beirut Medical Center, Beirut 11-0236, Lebanon
3
Department of Medicine, Ca’Granda Foundation IRCCS, University of Milan, Milan 20122, Italy
4
Department of Clinical Science and Community, University of Milan, Milan 20122, Italy
*
Author to whom correspondence should be addressed.
Received: 23 November 2017 / Revised: 17 December 2017 / Accepted: 20 December 2017 / Published: 20 December 2017
(This article belongs to the Special Issue Thalassemia in 2017)
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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. View Full-Text
Keywords: non-transfusion dependent thalassemia; iron overload; iron chelation therapy; serum ferritin; liver iron concentration non-transfusion dependent thalassemia; iron overload; iron chelation therapy; serum ferritin; liver iron concentration
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Bou-Fakhredin, R.; Bazarbachi, A.-H.; Chaya, B.; Sleiman, J.; Cappellini, M.D.; Taher, A.T. Iron Overload and Chelation Therapy in Non-Transfusion Dependent Thalassemia. Int. J. Mol. Sci. 2017, 18, 2778.

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