Iron Chelation Therapy in Beta Thalassemia

Authors

The Department of Pediatric*, Faculty of Medicine, Beni Suef University, Pharmaceutics Department**, Faculty of Pharmacy, Minia University and Clinical Pharmacy Department***, Faculty of Pharmacy, Beni Suef University

Abstract

Abstract Background: Iron accumulation is an inevitable conse-quence of chronic blood transfusions and results in serious complications in the absence of chelation treatment to remove excess iron. Desferioxamine reduces morbidity and mortality although the administration schedule of slow, parentral infu-sions several days each week limits compliance and negatively affects long-term outcome, so different strategies have been developed to overcome these problems such as deferiprone or deferasirox alone or dual chelator therapy. Aim of Study: Was to evaluate the effect of monotherapy and alternating therapy of iron chelators (deferioxamine, deferiprone, deferasirox) after six months of follow-up of regular administration of these iron chelators in hematology Clinic in pediatric Insurance Hospitals in Beni Suef. Patients and Methods: This study was carried out on 120 children with beta thalassemia major in hematology Clinic in pediatric Insurance Hospitals in Benisuef. They were divided into four groups. Group A: 30 patients received oral deferiprone (DFP) at 75mg/kg/day for 4 days/week and subcutaneous desferioxamine (DFO) at 40mg/kg/day for the other 3 days/ week for 6 months. Group B: 30 patients who received oral deferiprone only at 75mg/kg/day in 3 divided doses for 6 months. Group C: 30 patients who received subcutaneous desferoxamine only at 40mg/kg/day daily for 6 months. Group D: 30 patients who received oral desferasirox at a dose of 30mg/kg/day, single dose daily, taken on an empty stomach at least 30 minutes before food for 6 months. Results: There were highly significant reduction in serum ferritin levels and serum iron levels after chelation therapy in each studied group. There was also elevation in TIBC after chelation therapy in each studied group. The reduction of serum ferritin levels and serum iron levels and the elevation of TIBC were higher in group A (alternating) followed by group C (desferioxamine) followed by group D (deferasirox) and lastly group B (deferiprone). There was no statistically significant difference between the studied groups before and after chelation therapy. There was no significant difference as regard to urinary iron before chelation therapy in all studied group and also between the studied groups of patients after chelation therapy. But 24h urinary iron showed a significant difference in group A and insignificant difference in other groups of patients before and after chelation therapy. Conclusion: Alternating DFO/DFP has some significant advantages over DFO monotherapy; it can keep a balanced iron load, targets different iron pools & is well accepted by the patients. This approach is more appropriate for well-chelated patients, who have difficulties in continuing DFO monotherapy.