Sickle Cell Disease: Role of Oxidative Stress and Antioxidant Therapy

Sickle cell disease (SCD) is the most common hereditary disorder of hemoglobin (Hb), which affects approximately a million people worldwide. It characterized by single nucleotide substitution in ?-globin gene, leading to production abnormal sickle (HbS) with multi-system consequences. HbS polymerization primary event SCD. Repeated and depolymerization Hb causes oxidative stress that plays key role pathophysiology hemolysis, vessel occlusion following organ damage patients. For this reason, reactive oxidizing species (end)-products their reactions have been proposed as markers both tissue pro-oxidant status severity. Although more studies are needed clarify role, antioxidant agents shown be effective reducing pathological consequences preventing SCD, i.e., decreasing oxidant formation or repairing induced damage. An improved understanding will lead targeted therapies should prevent delay development complications patient population.