Abstract

Sickle erythrocytes are characterized by the presence of sickle hemoglobin (HbS), instead of normal one (Hb). HbS suffers recurrent polymerization/ de polymerization, it has tendency to autoxidise faster than Hb, leading to formation of hemi-chromes, and increased and unremitting pro oxidant generation. As consequence, sickle erythrocytes show a hyper-oxidative state that appears to be involved in the rupture of the metabolic homeostasis in this cell. This rupture causes a series of clinical complications in a vicious cycle in which many different oxidative processes play a central role in the patho physiology of sickle cell diseases. Thus, in this review, we briefly overview the implications of the HbS presence in the disruption of erythrocyte metabolic homeostasis, addressing the integration of some erythrocyte metabolic pathways, such as redox, glycolytic, pentose phosphate, methemoglobin reductive and Rapoport-Luebering ones.

Keywords: Hemoglobin S; Oxidative processes; Antioxidants; Metabolic integration