Oxidative stress in β-thalassaemia and sickle cell disease

• Hb autoxidation, Fenton reaction, NADPH oxidase: main sources of oxidants in RBCs.
• Intracellular oxidation: senescence/eryptosis/haemolysis, I.E., erythroid expansion.
• Extracellular oxidation: inflammation, coagulation, I/R injury, Fe overload.
• Protection in RBCs: antioxidant enzymes and molecules, PQC, FOXO3, HRI, NRF2, AHSP.
• Protection in circulation: haptoglobin, haemopexin, HO1.

Sickle cell disease and β-thalassaemia are inherited haemoglobinopathies resulting in structural and quantitative changes in the β-globin chain. These changes lead to instability of the generated haemoglobin or to globin chain imbalance, which in turn impact the oxidative environment both intracellularly and extracellularly. The ensuing oxidative stress and the inability of the body to adequately overcome it are, to a large extent, responsible for the pathophysiology of these diseases. This article provides an overview of the main players and control mechanisms involved in the establishment of oxidative stress in these haemoglobinopathies.

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