title: Rheology of red blood cells in patients with HbC disease.
abstract: Patients with hemoglobin C disease (CC) usually do not develop severe complications in comparison with individuals with sickle cell anemia (SS) or with sickle cell hemoglobin C disease (SC). The present study compared the hematological, biochemical, hemorheological and clinical characteristics of CC patients to those of SS, SC and healthy individuals (AA). Blood viscosity was measured at 225 s(-1) with a cone plate viscometer. The hematocrit-to-blood viscosity ratio (HVR), i.e. an index of red blood cell (RBC) oxygen transport effectiveness, was calculated. RBC deformability was determined at 30 Pa by ektacytometry, and RBC aggregation properties by syllectometry. CC and SC had higher blood viscosity and lower HVR than AA. Nevertheless, HVR was higher in CC compared to SS and tended to be higher than in SC. The CC group exhibited very rigid hyperchromic RBC compared to the three other groups. RBC aggregation abnormalities were observed in CC: low RBC aggregation index and high RBC aggregates strength. Despite these hemorheological abnormalities, CC never had hospitalized painful vaso-occlusive crisis or acute chest syndrome. In contrast, all of them had splenomegaly. Of note, 2 out of 7 CC developed retinopathy or otologic disorders. Whether the blood hyperviscosity and decreased RBC deformability are responsible for these complications is unknown. The higher oxygen transport effectiveness (i.e., HVR) of CC compared to SS is probably at the origin of the very low risk of medical complication in this population.
authors.affiliation: Unité Transversale de la Drépanocytose, CHU de Pointe-à-Pitre, 97159 Pointe-à-Pitre, Guadeloupe.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Lemonne
authors.affiliation: Unité Transversale de la Drépanocytose, CHU de Pointe-à-Pitre, 97159 Pointe-à-Pitre, Guadeloupe.
authors.forename: Marie
authors.initials: M
authors.lastname: Billaud
authors.affiliation: Inserm U 1134, Université des Antilles et de la Guyane, 97159 Pointe-à-Pitre, Guadeloupe.|Laboratory of Excellence GR-Ex, Paris, France.|Laboratoire ACTES-EA3596, Université des Antilles et de la Guyane, 97157 Pointe-à-Pitre, Guadeloupe.
authors.forename: Xavier
authors.initials: X
authors.lastname: Waltz
authors.affiliation: Inserm U 1134, Université des Antilles et de la Guyane, 97159 Pointe-à-Pitre, Guadeloupe.|Laboratory of Excellence GR-Ex, Paris, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Romana
authors.affiliation: Inserm U 1134, Université des Antilles et de la Guyane, 97159 Pointe-à-Pitre, Guadeloupe.|Laboratory of Excellence GR-Ex, Paris, France.
authors.forename: Régine
authors.initials: R
authors.lastname: Hierso
authors.affiliation: Unité Transversale de la Drépanocytose, CHU de Pointe-à-Pitre, 97159 Pointe-à-Pitre, Guadeloupe.|Inserm U 1134, Université des Antilles et de la Guyane, 97159 Pointe-à-Pitre, Guadeloupe.
authors.forename: Maryse
authors.initials: M
authors.lastname: Etienne-Julan
authors.affiliation: Inserm U 1134, Université des Antilles et de la Guyane, 97159 Pointe-à-Pitre, Guadeloupe.|Laboratory of Excellence GR-Ex, Paris, France.|Institut Universitaire de France, Paris, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Connes
mesh_terms: D000328:Adult; D001809:Blood Viscosity; D004906:Erythrocyte Count; D004907:Erythrocyte Deformability; D004912:Erythrocytes / Q000378:metabolism*; D005260:Female; D006445:Hemoglobin C Disease / Q000097:blood* / Q000473:pathology; D018056:Hemorheology; D006801:Humans; D008297:Male; D008875:Middle Aged
keywords: HbC disease; blood viscosity; red blood cell deformability; sickle cell disease