Abstract
Background: Thalassemia is an autosomal recessive hemoglobin disorder requiring life-long blood transfusion causing iron overload. Combination therapy of oral iron chelators: Deferiprone (DFP) with Deferasirox (DFX) is an efficacious and safe modality to reduce serum ferritin in multi-transfused thalassemic children. This study was carried out to evaluate the effect of Deferiprone versus Deferiprone with Deferasirox in iron chelation therapy of transfusion dependent thalassemia children.
Methodology: A cross sectional observational study was done in Pediatric Hematology and Oncology, Dhaka Medical College Hospital and Bangladesh Thalassemia Hospital in Dhaka from January to June 2018. Children with transfusion dependent β-thalassemia major and E-β-thalassemia having serum ferritin level >1000 ng/ml between 3 to 12 years of age were studied.
Results: Demographic profile of 60 cases demonstrated that there are no significant differences in age, sex and weight between DFP-monotherapy and DFP-DFX-combination groups. Initial mean serum ferritin level was 3397.48±774.48 ng/ml in DFP-monotherapy group and 3413.70±1114.05ng/ml in DFP-DFX combination group. After treatment mean serum ferritin level at 6th month was 2730.63±839.91 ng/ml in DFP-monotherapy group and 1654.20±934.90 ng/ml in DFP-DFX-combination group which shows rapid reduction of serum ferritin level in DFP-DFX-combination group. Mean dose of drugs was 72.59(±3.76) mg/kg/ day of DFP in monotherapy group and 68.68(±4.84) mg/kg/ day of DFP and 27.98(±2.13) mg/kg/ day of DFX in combination group. 12 (40.0%) patients had arthralgia in DFP-monotherapy group and 5(16.7%) patients had vomiting in DFP-DFX-combination group. No abnormalities seen in liver and renal function tests.
Conclusion: Deferiprone alone is capable in significant excretion of iron from the body in transfusion dependent thalassemia major patients. But combination therapy with Deferiprone and Deferasirox is more significantly effective in iron chelation and drug induced related complications.
Keywords: Thalassemia major, iron overload, effects of combined oral iron chelation therapy.
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Corresponding Author
Dr Syeda Jarka Jahir
Department of Pediatrics, Dhaka Medical College Hospital, Dhaka, Bangladesh
DOI: https://dx.doi.org/10.18535/jmscr/v6i9.57
