Blood rheology in children with the S/β+-thalassemia syndrome

Abstract

The aim of the present study was to compare blood rheological parameters between children with homozygous sickle cell disease (SS), sickle cell SC disease or S/β+-thalassemia syndrome, and healthy children (AA) and to test the associations between blood rheology and the clinical severity in S/β+-thalassemia. Sixty-two SS, 14 SC, 11 S/β+-thalassemia and 12 healthy children participated in this study. Blood viscosity was measured with a cone-plate viscometer at 225 s-1. Red blood cell (RBC) deformability was measured by ektacytometry and RBC aggregation, by syllectometry. Nitric oxide and nitrotyrosine levels were determined for each child. While most of the hematological parameters were not different between SC and S/β+-thalassemia children, we demonstrated that SC patients had lower RBC deformability and aggregation than S/β+ individuals. Nitrotyrosine level, which indicates peroxynitrite production, was similar and lower in both healthy and S/β+ compared to SS children. However, S/β+-thalassemia children who experienced vaso-occlusive crises (VOC) in the 2 previous years had lower NOx and higher nitrotyrosine levels than those who never had VOC within the same period. These findings suggest that vascular function could be impaired in the most severe S/β+-thalassemia children compared to the less severe one.

Keywords: Beta-thalassemia; nitric oxide; red blood cell deformability; sickle cell disease.