Redox Balance in β-Thalassemia and Sickle Cell Disease: A Love and Hate Relationship

Abstract

β-thalassemia and sickle cell disease (SCD) are inherited hemoglobinopathies that result in both quantitative and qualitative variations in the β-globin chain. These in turn lead to instability in the generated hemoglobin (Hb) or to a globin chain imbalance that affects the oxidative environ-ment both intracellularly and extracellularly. While oxidative stress is not among the primary etiol-ogies of β-thalassemia and SCD, it plays a significant role in the pathogenesis of these diseases. Different mechanisms exist behind the development of oxidative stress; the result of which is cyto-toxicity, causing the oxidation of cellular components that can eventually lead to cell death and organ damage. In this review, we summarize the mechanisms of oxidative stress development in β-thalassemia and SCD and describe the current and potential antioxidant therapeutic strategies. Fi-nally, we discuss the role of targeted therapy in achieving an optimal redox balance.