Asunto(s)
Anemia de Células Falciformes/tratamiento farmacológico , Preescolar , Ciclofosfamida/uso terapéutico , Hemoglobina E/efectos de los fármacos , Hemólisis/efectos de los fármacos , Humanos , Inmunoglobulinas Intravenosas , Inmunosupresores/uso terapéutico , Masculino , Reticulocitos/efectos de los fármacos , Esplenectomía , Síndrome , Talasemia beta/tratamiento farmacológicoRESUMEN
OBJECTIVES: To assess efficacy and safety of oral iron chelating agent deferiprone (DFP) in patients with beta thalassemia and hemoglobin E-beta thalassemia. DESIGN: Non-randomized study. SETTING: Hematology Out-Patient Department. SUBJECTS: Forty-one patients of beta thalassemia and hemoglobin E-beta thalassemia. INTERVENTIONS: DFP was given to 20 patients, 10 patients of beta thalassemia and 10 with hemoglobin E-beta thalassemia; the rest were taken as controls. RESULTS: A significant fall in serum ferritin was observed in the study group along with rise in urinary iron excretion (p < 0.05). Adverse effects of DFP were nausea and vomiting (30%), significant arthropathy requiring stopping of the drug (30%), and reversible neutropenia in one patient. All these complications could be managed easily with medical supervision and no death or permanent disability was seen. CONCLUSIONS: DFP is an effective and fairly well tolerated oral iron chelating agent. The side effects that occur can be tackled easily if monitored properly.