Iron overload in thalassaemia intermedia : reassessment of iron chelation strategies

Thalassaemia intermedia (TI) is a syndrome marked by its diverse underlying genetic basis although its pathophysiology remains unclear, particularly regarding the nature of iron loading and toxicity. It is, however, evident that there are key differences from the extensively studied thalassaemia major (TM) population and caution is required when assessing iron load based on serum ferritin values, as this approach is known to underestimate the true extent of iron loading in patients with TI. Although effective iron chelation therapy has been available for many years, studies in TI-specific populations are rare and evidence suggests that management of iron levels may be less rigorous than in patients with TM and other chronic anaemias. Better understanding of the need to assess and treat iron overload in both transfused and non-transfused TI patients is clearly required.