Vitamin E Supplementation for Children With Transfusion Dependent Beta Thalassemia on Different Iron Chelation Regimen

Vitamin E Supplementation for Children With Transfusion Dependent Beta Thalassemia on Different Iron Chelators

repeated transfusions are the mainstay of disease management in most patients with transfusion dependent beta thalassemia.iron overload predispose to oxidative stress and tissue injury.

oxidative stress play important role in pathogenesis of anemia in beta thalassemia.

vitamin E is often depleted in thalassemia patients.

oxidative stress status is very important in thalassemic patients and explains the different manifestations in thalassemic patients.

vitamin E is fat soluble vitamin shown to reduce the oxidative stress in thalassemia and to reduce lipid peroxidation of red cell membranes.

therefore, this study shows the safety of oral vitamin E as adjuvant therapy to three iron chelators : desferoxamine,deferiprone and deferasirox in moderately iron overloaded children and adolescents with transfusion dependent beta thalassemia and its relation to iron overload over one year.

Inclusion Criteria:

• Beta thalassemia patients on regular packed red blood cells transfusion,with vitamin E deficiency
• serum ferritin 1000-2500 ug/l
• cardiacT2*>10ms and ejection fraction >55%

Exclusion Criteria:

• Diabetes,active hepatitis(serum transaminases >2 times ULN)
• renal impairment (serum creatinine>2times ULN)
• participation in previous investigational drug study with 3 months preceding screening supplementation with antioxidants or vitamins within 3 months prior to the study and patients with known allergy to vitamin