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.