Glutathione metabolism of erythrocytes in the paroxysmal nocturnal hemoglobinuria

Abstract

Introduction. Oxidative stress (OS) is involved in the development of vascular inflammation and hemostasis. The aim. We hypothesized that GSH-dependent antioxidant defense system may be affected in paroxysmal nocturnal hemoglobinuria (PNH), causing exacerbation of inflammation and activation of intravascular coagulation and fibrinolysis (AIVCF). Methods. The study involved 63 patients with PNH and 75 healthy people. The concentration of GSH and the activity of glutathione peroxidase (GP), glutathione reductase (GR) and superoxide dismutase (SOD) have been determined in the erythrocytes. Results. We have demonstrated the inhibition of glutathione metabolism and signs of OS, expressed in a significant reduction in levels of GSH and decreased activity of antioxidant enzymes GP, GR and SOD in erythrocytes when compared PNH patients with healthy people. Spearman's test showed significant positive correlation between the activity of GR and GP (R = 0.376; p<0.01) in red blood cells, as well as a weak negative correlation (R = -0.338; p<0.05) between GP activity in erythrocytes and the concentration of D-dimer in the blood plasma in patients with PNH. A positive (R = 0.626; p<0.001) correlation was found between GSH in erythrocytes and plasma concentrations of CRP at CRP concentration values in the range of reference interval. Conclusion. The results are consistent with previously published data on disorders of glutathione metabolism in PNH. The indicators GSH and GP in erythrocytes can be considered as potential markers of AIVCF and vascular inflammation in PNH.