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

Kizzy-Clara Cita (1) , Laurent Brureau (2) , Nathalie Lemonne (3) , Marie Billaud (4) , Philippe Connes (5, 6) , Séverine Ferdinand (1, 7) , Benoit Tressières (8) , Vanessa Tarer (9) , Maryse Etienne-Julan (3) , Pascal Blanchet (2) , Jacques Elion (10) , Marc Romana (7)

1 Observatoire Régional de la Santé de la Guadeloupe
2 Irset - Institut de recherche en santé, environnement et travail
3 CHU Pointe-à-Pitre/Abymes [Guadeloupe]
4 CRCTB - Centre de recherche Cardio-Thoracique de Bordeaux [Bordeaux]
5 UAG - Université des Antilles et de la Guyane
6 LIBM - Laboratoire Interuniversitaire de Biologie de la Motricité
7 BIGR (UMR_S_1134 / U1134) - Biologie Intégrée du Globule Rouge
8 CIC - Antilles Guyane - Centre d'Investigation Clinique Antilles-Guyane
9 Centre de référence maladies rares pour la drépanocytose aux Antilles-Guyane
10 Insern, UMR 763, Hôpital Robert Debré

Objectives To investigate the association between priapism in men with sickle cell anemia (SCA) and hemorheological and hemolytical parameters. Materials and Methods 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. Results 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. Conclusion 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.

Mots clés: alpha-thalassemia; risk-factors; disease; hydroxyurea; children; complications; prevalence; frequency; viscosity; rheology

Domaines: Santé

Kizzy-Clara Cita, Laurent Brureau, Nathalie Lemonne, Marie Billaud, Philippe Connes, et al.. Men with Sickle Cell Anemia and Priapism Exhibit Increased Hemolytic Rate, Decreased Red Blood Cell Deformability and Increased Red Blood Cell Aggregate Strength. PLoS ONE, 2016, 11 (5), pp.e0154866. ⟨10.1371/journal.pone.0154866⟩. ⟨hal-01390971⟩