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

Issue title: Special Issue in Honour of the Editor-in-Chief Prof. Dr. Ing. Friedrich Jung on his 70th Birthday

Article type: Research Article

Authors: Renoux, Céline^{a; b; c} | Joly, Philippe^{a; b; c} | Gauthier, Alexandra^{a; b; d} | Pialoux, Vincent^{a; b; e} | Romanet-Faes, Camille^{a; b} | Bertrand, Yves^d | Garnier, Nathalie^d | Cuzzubbo, Daniela^d | Cannas, Giovanna^{a; b; f} | Connes, Philippe^{a; b; e; *}

Affiliations: [a] Univ Lyon, Université Claude Bernard Lyon 1, Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Villeurbanne, France | [b] Laboratoire d’Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France | [c] Laboratoire de biochimie des pathologies érythrocytaires, Centre de Biologie Est, Hospices Civils de Lyon, France | [d] Institut d’Hématologie et d’Oncologie Pédiatrique (IHOPe), Hospices Civils de Lyon, France | [e] Institut Universitaire de France (IUF), Paris, France | [f] Clinique de Médecine Ambulatoire/Hématologie Hôpital Edouard Herriot, Lyon, France

Correspondence: [*] Corresponding author: Philippe Connes, PhD, Univ Lyon, Université Claude Bernard Lyon 1, Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Villeurbanne, France. E-mails: [email protected] and [email protected].

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, sickle cell disease, red blood cell deformability, nitric oxide

DOI: 10.3233/CH-189119

Journal: Clinical Hemorheology and Microcirculation, vol. 69, no. 1-2, pp. 207-214, 2018