PREVENTION OF HEART-DISEASE BY SUBCUTANEOUS DESFERRIOXAMINE IN PATIENTS WITH THALASSEMIA MAJOR

Background: Cardiac siderosis from transfused iron remains the major cause of death in thalassaemia major, despite iron chelation therapy with desferrioxamine. Aims: Our aim was to determine the nature and extent of cardiac involvement and its relationship to desferrioxamine use in a group with thalassaemia major. Methods: We reviewed 76 patients with thalassaemia major and performed multiple logistic regression to analyse factors affecting cardiac involvement. Factors studied included: patient sex, age, haemoglobin, serum ferritin, total transfusions, liver iron, duration of desferrioxamine use, electrocardiograms, echocardiograms and compliance to desferrioxamine treatment. Results: Thirty-seven patients developed heart disease. They were older (p < 0.001), began desferrioxamine later (p < 0.001), had more liver iron (p = 0.014), higher serum ferritin levels (p = 0.023) and received more blood (p = 0.018). Compared to those with optimal compliance the odds of developing heart disease were increased 10.7 times in fair compliers (p < 0.001) and 5.1 times in poor compliers (p = 0.016). However, there was no significant difference between those with fair and poor compliance. After multivariate analysis only compliance (p = 0.02) and age at desferrioxamine onset (p = 0.004) remained significant. Compliance was inversely related to liver iron (p < 0.001), serum ferritin (p < 0.001) and age at desferrioxamine commencement (p < 0.001). Conclusions: We conclude that late commencement of desferrioxamine and noncompliance are associated with greater iron loading and an increased risk of heart disease.