Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hematol. Aug 6, 2017; 6(3): 55-61
Published online Aug 6, 2017. doi: 10.5315/wjh.v6.i3.55
Oxidative alterations in sickle cell disease: Possible involvement in disease pathogenesis
Yesim Oztas, Ahmet Yalcinkaya
Yesim Oztas, Ahmet Yalcinkaya, Department of Medical Biochemistry, Faculty of Medicine, Hacettepe University, 06100 Ankara, Turkey
Author contributions: Oztas Y designed the concept of the manuscript and wrote it; Yalcinkaya A contributed in writing some sections.
Conflict-of-interest statement: There is no conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Yesim Oztas, MD, PhD, Department of Medical Biochemistry, Faculty of Medicine, Hacettepe University, Sihhiye, 06100 Ankara, Turkey.
Telephone: +90-312-3051652 Fax: +90-312-3245885
Received: January 8, 2017
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.

Keywords: Oxidative stress, Sickle cell disease, Iron, Protein oxidation, Carbonyl group, Sulfhydryl group, Low-density lipoprotein, High-density lipoprotein

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.