Oxidative stress and sickle cell disease

Sickle cell disease (SCD) embraces a group of genetic hemolytic disorders associated with high morbidity and mortality. SCD is characterized by a complex pathophysiology initiated by hemoglobin S (HbS) polymerization that triggers a cascade of pathological events, including vaso-occlusion episodes, hemolysis, endothelial dysfunction, inflammation, hypercoagulability, reperfusion injury, and hypoxemia, leading to devastating clinical manifestations. Although SCD is one of the first disorders to be clearly defined at the molecular level, the genetic understanding of the basis for the disease expression variability is still not fully explained. In this intriguing scenario, oxidative stress plays a major role because it acts as both causing and being caused by SCD complications.