Published online Aug 6, 2017. doi: 10.5315/wjh.v6.i3.55
Peer-review started: January 12, 2017
First decision: February 16, 2017
Revised: April 26, 2017
Accepted: May 21, 2017
Article in press: May 22, 2017
Published online: August 6, 2017
Sickle cell disease (SCD) is the first molecular disease in the literature. Although the structural alteration and dysfunction of the sickle hemoglobin (HbS) are well understood, the many factors modifying the clinical signs and symptoms of the disease are under investigation. Besides having an abnormal electrophoretic mobility and solubility, HbS is unstable. The autooxidation rate of the abnormal HbS has been reported to be almost two times of the normal. There are two more components of the oxidative damage in SCD: Free radical induced oxidative damage during vaso-occlusion induced ischemia-reperfusion injury and decreased antioxidant capacity in the erythrocyte and in the circulation. We will discuss the effects of oxidative alterations in the erythrocyte and in the plasma of SCD patients in this review.
Core tip: Oxidative alterations in the plasma and erythrocyte of sickle cell disease may indicate disease progression and phenotype. Detected oxidative modifications may be used as disease markers. Novel drugs targetting oxidative damage of plasma and cellular components may be important as promising therapeutic options.