Journal Article

Men with Sickle Cell Anemia and Priapism Exhibit Increased Hemolytic Rate, Decreased Red Blood Cell Deformability and Increased Red Blood Cell Aggregate Strength

Lemonne, Nathalie, Ferdinand, Séverine, Tarer, Vanessa, Romana, Marc, Tressières, Benoit, Etienne-Julan, Maryse, Brureau, Laurent, Connes, Philippe, Billaud, Marie, Cita, Kizzy-Clara, Blanchet, Pascal, Elion, Jacques

2016

Overview

To investigate the association between priapism in men with sickle cell anemia (SCA) and hemorheological and hemolytical parameters. Fifty-eight men with SCA (median age: 38 years) were included; 28 who had experienced priapism at least once during their life (priapism group) and 30 who never experienced this complication (control group). Twenty-two patients were treated with hydroxycarbamide, 11 in each group. All patients were at steady state at the time of inclusion. Hematological and biochemical parameters were obtained through routine procedures. The Laser-assisted Optical Rotational Cell Analyzer was used to measure red blood cell (RBC) deformability at 30 Pa (ektacytometry) and RBC aggregation properties (laser backscatter versus time). Blood viscosity was measured at a shear rate of 225 s-1 using a cone/plate viscometer. A principal component analysis was performed on 4 hemolytic markers (i.e., lactate dehydrogenase (LDH), aspartate aminotransferase (ASAT), total bilirubin (BIL) levels and reticulocyte (RET) percentage) to calculate a hemolytic index. Compared to the control group, patients with priapism exhibited higher ASAT (p = 0.01), LDH (p = 0.03), RET (p = 0.03) levels and hemolytic indices (p = 0.02). Higher RBC aggregates strength (p = 0.01) and lower RBC deformability (p = 0.005) were observed in patients with priapism compared to controls. After removing the hydroxycarbamide-treated patients, RBC deformability (p = 0.01) and RBC aggregate strength (p = 0.03) were still different between the two groups, and patients with priapism exhibited significantly higher hemolytic indices (p = 0.01) than controls. Our results confirm that priapism in SCA is associated with higher hemolytic rates and show for the first time that this complication is also associated with higher RBC aggregate strength and lower RBC deformability.

• Publisher: Public Library of Science,Public Library of Science (PLoS)
• Subject: Adult / Anemia / Anemia, Sickle Cell - blood / Anemia, Sickle Cell - pathology / Aspartate aminotransferase / Backscatter / Backscattering / Bilirubin / Biology and Life Sciences / Biomarkers - blood / Blood / Blood Viscosity - physiology / Deformability / Deformation / Ecology, environment / Erythrocyte Aggregation - physiology / Erythrocyte Deformability - physiology / Erythrocytes / Erythrocytes - pathology / Formability / Genital diseases / Health / Hematology / Hemolysis - physiology / Hemorheology - physiology / Humans / Hydroxyurea / L-Lactate dehydrogenase / Laboratories / Lactate dehydrogenase / Lactic acid / Lasers / Life Sciences / Male / Medicine and Health Sciences / Men / Middle Aged / Parameters / Patients / Penis / Physical Sciences / Priapism - blood / Priapism - pathology / Principal components analysis / Prospective Studies / Research and Analysis Methods / Reticulocytes - pathology / Rheology / Shear rate / Sickle cell anemia / Sickle cell disease / Strength / Viscosity