Blood rheological abnormalities in sickle cell anemia

Issue title: Sickle Cell Disease

Guest editors: P. Connes

Article type: Research Article

Authors: Connes, Philippe^{a; b; c; *} | Renoux, Céline^{a; b; d} | Romana, Marc^{b; e} | Abkarian, Manouk^f | Joly, Philippe^{a; b; d} | Martin, Cyril^{a; b} | Hardy-Dessources, Marie-Dominique^{b; e} | Ballas, Samir K.^g

Affiliations: [a] Laboratoire LIBM EA7424, Team“Vascular Biology and Red Blood Cell”, Université Claude Bernard Lyon 1, University of Lyon, 69100 Villeurbanne, France | [b] Laboratory of Excellence GR-Ex « The red cell: from genesis to death », PRES Sorbonne Paris Cité, 75015, Paris, France | [c] Institut Universitaire de France, Paris, France | [d] Laboratoire de biochimie des pathologies érythrocytaires, Centre de Biologie Est, Hospices Civils de Lyon, France | [e] Inserm UMR 1134, Hôpital Ricou, CHU de Pointe-à-Pitre, 97157 Pointe-à-Pitre, Guadeloupe | [f] CNRS UMR 5048, Université de Montpellier, Centre de Biochimie Structurale, 34090 Montpellier, France | [g] Department of Medicine, Cardeza Foundation for Hematologic Research, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA

Correspondence: [*] Corresponding author: Philippe Connes, PhD, Laboratoire LIBM EA7424, Team“Vascular Biology and Red Blood Cell”, Université Claude Bernard Lyon 1, 69100 Villeurbanne, France. E-mails: pconnes@yahoo.fr/philippe.connes@univ-lyon1.fr.

Abstract: This review focuses on the contribution of abnormal blood rheology in the pathophysiology of sickle cell anemia (SCA). SCA is characterized by a reduction of red blood cell (RBC) deformability but this reduction is very heterogeneous among patients. Recent works have shown that patients with the lowest RBC deformability (measured by ektacytometry) have enhanced hemolysis and would be more prone to develop several complications such as priapism, leg ulcers and glomerulopathy. In contrast, patients with the highest deformability, and not under hydroxyurea therapy, seem to develop more frequently vaso-occlusive like events. Although less studied, RBC aggregation properties are very different between SCA and healthy individuals and it was demonstrated that increased RBC aggregates strength could be involved in some complications. Finally, several studies have established that the vascular system of SCA patients could not fully compensate any increase in blood viscosity because of the loss of vascular reactivity, which may result in vaso-occlusive crises.

Keywords: Sickle cell anemia, blood viscosity, red blood cell deformability, red blood cell aggregation

DOI: 10.3233/CH-189005

Journal: Clinical Hemorheology and Microcirculation, vol. 68, no. 2-3, pp. 165-172, 2018