In search of the optimal iron chelation therapy for patients with thalassemia major.

摘要

Despite the fact that iron chelation therapy has been available for more than forty years, iron cardiomy-opathy is the most common cause of death in patients with transfusion dependent anemia.1'2 In the last decade, however, such deaths have been significantly reduced.This improvement has been attributed in part to greater iron chelation options and the ability to recognize pre-clinical cardiac iron deposition by Magnetic Resonance Imaging studies (MRI). Patients with low cardiac T2*, as measured by MRI, have a significant risk of developing overt heart failure over a 12-month period if changes to their chelation regime are not made (Figure l).s Surrogate markers used in the past, such as ferritin and liver iron concentration for predicting cardiac risk, have been shown not to be particularly predictive of the degree of cardiac iron load (Figures 2A and B) with an r value of less than 0.5 for both.7 This indicates how valuable MRI measurements are. Optimal chelation therapy remains a controversial topic.