Effect of combined desferal/ defriprone therapy on iron overload in beta thalassemic children

ABSTRACT

Management of B-thalassemia major with regular blood transfusion and iron chelation therapy with desferrioxamine [DFO] has markedly improved the prognosis of the disease but some patients continue to develop iron-related complications and to die despite the use of DFO. Deferiprone [DFP] is suggested to be the most promising oral iron chelator under development. The present study was conducted on 60 pediatric patients with Beta-thalassemia major to evaluate the effectiveness of combined therapy with DFO and DFP compared to DFO therapy alone. The patients were evaluated clinically with complete history taking, routine laboratory investigations, doppler echocardiography, serum ferritin and serum cardiac troponin T [cTnT] estimation. The results showed significant improvement in hemodynamic parameters and significant decrease in serum cTnT and decrease in serum ferritin post-treatment with DFO and combined DFO/DFP when compared with pre-treatment values of the patients. Combination therapy was more effective than DFO therapy alone in the improvement of hemodynamic parameters, reduction of the myocardial damage indicator [cTnT] and reduction of serum ferritin. The authors recommend the use of combined DFO/DFP therapy in the management of transfusional iron overload in beta-thalassemia major