COMPARATIVE STUDY OF THERAPEUTIC VALUES OF DIFFERENT IRON CHELATING AGENTS IN CHILDREN WITH BETA THALASSEMIA MAJOR

Backgrounds: Beta thalassemia is hereditary blood disorder characterized by reduced or absent beta chains of hemoglobin resulting in imbalanced globin chain synthesis with early destruction of RBCs and anemia. Patients with thalassemia major become transfusion-dependent with subsequent iron overload. Effective and convenient iron chelation therapy remains one of the main targets of management of thalassemia major. Objectives: The aim of this work was to compare the efficacy of different iron chelating agents in treatment of iron overload in children with beta thalassemia major. Patients and Methods: The current study was conducted on 120 children with beta thalassemia major with serum ferritin level above 1000 ng/ml who were divided into 4 groups: Group A: 30 patients were treated with 8 hours intravenous infusion of desferrioxamine, 40 mg/kg/day, 6 days per week for 6 months. Group B: 30 patients were treated with subcutaneous infusion of desferrioxamine, 40 mg/kg/day, 6 days per week 8-12 hours per day at night using desferal pumb for 6 months. Group C: 30 patients were treated with oral deferiprone 75mg/kg/day in three divided doses daily for 6 months. Group D: 30 patients were treated with oral deferasirox 30 mg/kg/day in single dose on empty stomach daily for 6 months. All thalassemic patients were subjected to complete history taking, thorough clinical examination and laboratory investigations including complete blood count, serum ferritin, iron, TIBC, liver and kidney functions. Results: There were significant reductions in serum ferritin and serum iron after treatment in all studied groups with the highest reduction in serum ferritin and serum iron in group A , group B , group D and group C but without statistically significant differences between the four studied groups before and after chelation therapy. There were no significant differences in the mean values of the parameters of CBC, liver enzymes and kidney function between the studied groups before and after chelation therapy. Conclusion: Iron chelators are cornerstone in treatment of iron overload in children with beta thalassemia. IV desferrioxamine therapy is more effective in treatment of iron overload compared with SC desferrioxamine, oral deferasirox and oral deferiprone with no statistically significant difference. Recommendations: IV desferrioxamine therapy should be considered for the treatment of iron overloaded in thalassemic patients. Further studies on large number of patients and for long duration are required to clarify the side effects of long term use of iron chelating agents.