The role of blood rheology in sickle cell disease.
Studies performed in the last decades have highlighted the need to better understand the contribution of the endothelium, vascular function, oxidative stress, inflammation, coagulation, hemolysis and vascular adhesion mechanisms to the pathophysiology of acute vaso-occlusive like events and chronic organ damages in sickle cell disease (SCD). Although SCD is a hemorheological disease, a few works focused on the contribution of blood viscosity, plasma viscosity, red blood cell deformability and aggregation in the pathophysiology of SCD. After a brief description of basic hemorheology, the present review focuses on the role of the hemorheological abnormalities in the causation of several SCD complications, mainly in sickle cell anemia and hemoglobin (Hb) SC disease. Several genetic and cellular modulators of blood rheology in SCD are discussed, as well as unresolved questions and perspectives.
Inserm UMR 1134, Hôpital Ricou, CHU de Pointe-à-Pitre, 97157 Pointe-à-Pitre, Guadeloupe; Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, 75015 Paris, France; Institut Universitaire de France, Paris, France; Laboratoire CRIS EA647, Section "Vascular Biology and Red Blood Cell", Université Claude Bernard Lyon 1, 69100 Villeurbanne, France. Electronic address: pconnes@yahoo.fr.
Philippe
P
Connes
Division of Cardiology, Emory University School of Medicine, Atlanta, GA, USA; Section of Cardiology, Atlanta VA Medical Center, Decatur, GA, USA.
Tamas
T
Alexy
Children's Hospital Los Angeles, Division of Cardiology, USA.
Jon
J
Detterich
Inserm UMR 1134, Hôpital Ricou, CHU de Pointe-à-Pitre, 97157 Pointe-à-Pitre, Guadeloupe; Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, 75015 Paris, France.
Marc
M
Romana
Inserm UMR 1134, Hôpital Ricou, CHU de Pointe-à-Pitre, 97157 Pointe-à-Pitre, Guadeloupe; Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, 75015 Paris, France.
Marie-Dominique
MD
Hardy-Dessources
Department of Medicine, Cardeza Foundation for Hematologic Research, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
Samir K
SK
Ballas
D000755:Anemia, Sickle Cell / Q000097:blood* / Q000150:complications / Q000235:genetics / Q000378:metabolism; D001777:Blood Coagulation; D001809:Blood Viscosity; D004907:Erythrocyte Deformability; D006400:Hematocrit; D006461:Hemolysis; D018056:Hemorheology; D006801:Humans; D009154:Mutation; D018384:Oxidative Stress; D011379:Prognosis
Blood viscosity; Red blood cell aggregation; Red blood cell deformability; Sickle cell disease; Vaso-occlusive crises