Study The Effect Of Combination Iron Chelation Therapy In Transfusion Dependent Β Thalassemia Major

Abstract

Background and aims: Due to the availability of safe blood transfusions even at the village and district levels lead to the increased survival in transfusion dependent thalassemic children but at the same time it has leads increase iron overload and its complication in these patients. Deferasirox is the most commonly used iron chelation agent but in many children it is not able to decrease S. ferritin level to desired level hence require addition of other drug.

Material and method:A prospective analytical study was carried out to assess the effect of combination iron chelation therapy on children with transfusion-dependent beta thalassemia major children age between 6-18 years of age who are on Deferasiroxmonotherapy and S.ferritin>2500 ng/dl. All patients who were enrolled in the  study were started on combination iron chelation therapy with Deferiprone at a dose of 75 -100 mg/kg was added with Deferasirox after baseline investigations like S.Ferritin  and growth parameters like Height, weight and Sexual maturity rating (SMR) were done every 3 monthly and asked for regular follow up for blood transfusion.

Results –Statistically significant lower mean serum  ferritin values were  observed when the combination iron chelation therapy was used as compared to deferasiroxmonotherapy with p value 0.0022(p value<0.05) . There is no statistically significant difference in the height,weight and pubertal parameters were observed at the end of the study.

Conclusion- Combination iron chelation therapy with deferasirox and deferiprone is more effective than monotherapy with deferasirox for reducing serum ferritin level in children with β thalassemia major in age between 6-18 years with serum ferritin >2500 ng/ml with deferasiroxdose > 36mg/kg/day. Combination iron chelation therapy is relatively safer with minimal transient side effect.