title: Men with Sickle Cell Anemia and Priapism Exhibit Increased Hemolytic Rate, Decreased Red Blood Cell Deformability and Increased Red Blood Cell Aggregate Strength.
abstract: 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.
authors.affiliation: Inserm UMR 1134, Université des Antilles, Pointe à Pitre, Guadeloupe.|Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), COMUE Sorbonne Paris Cité, Paris, France.
authors.forename: Kizzy-Clara
authors.initials: KC
authors.lastname: Cita
authors.affiliation: CHU de Pointe-à-Pitre, Service d'Urologie, Pointe-à-Pitre, Guadeloupe.|Inserm, U1085-IRSET, Pointe-à-Pitre, Guadeloupe, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Brureau
authors.affiliation: CHU de Pointe-à-Pitre, Unité Transversale de la Drépanocytose, Centre de référence maladies rares de la drépanocytose aux Antilles-Guyane, Pointe-à-Pitre, Guadeloupe.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Lemonne
authors.affiliation: CHU de Pointe-à-Pitre, Unité Transversale de la Drépanocytose, Centre de référence maladies rares de la drépanocytose aux Antilles-Guyane, Pointe-à-Pitre, Guadeloupe.
authors.forename: Marie
authors.initials: M
authors.lastname: Billaud
authors.affiliation: Inserm UMR 1134, Université des Antilles, Pointe à Pitre, Guadeloupe.|Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), COMUE Sorbonne Paris Cité, Paris, France.|Institut Universitaire de France, Paris, France.|Université Claude Bernard Lyon 1, COMUE Lyon, Laboratoire LIBM EA 7424, Team "Vascular Biology and red blood cell", Lyon, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Connes
authors.affiliation: Inserm UMR 1134, Université des Antilles, Pointe à Pitre, Guadeloupe.|Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), COMUE Sorbonne Paris Cité, Paris, France.
authors.forename: Séverine
authors.initials: S
authors.lastname: Ferdinand
authors.affiliation: Inserm, CHU de Pointe-à-Pitre, Centre d'Investigation Clinique Antilles Guyane CIC 14-24, Guadeloupe, France.
authors.forename: Benoit
authors.initials: B
authors.lastname: Tressières
authors.affiliation: CHU de Pointe-à-Pitre, Unité Transversale de la Drépanocytose, Centre de référence maladies rares de la drépanocytose aux Antilles-Guyane, Pointe-à-Pitre, Guadeloupe.
authors.forename: Vanessa
authors.initials: V
authors.lastname: Tarer
authors.affiliation: CHU de Pointe-à-Pitre, Unité Transversale de la Drépanocytose, Centre de référence maladies rares de la drépanocytose aux Antilles-Guyane, Pointe-à-Pitre, Guadeloupe.
authors.forename: Maryse
authors.initials: M
authors.lastname: Etienne-Julan
authors.affiliation: CHU de Pointe-à-Pitre, Service d'Urologie, Pointe-à-Pitre, Guadeloupe.|Inserm, U1085-IRSET, Pointe-à-Pitre, Guadeloupe, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Blanchet
authors.affiliation: Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), COMUE Sorbonne Paris Cité, Paris, France.|Inserm U 1134, Paris, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Elion
authors.affiliation: Inserm UMR 1134, Université des Antilles, Pointe à Pitre, Guadeloupe.|Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), COMUE Sorbonne Paris Cité, Paris, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Romana
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000473:pathology; D015415:Biomarkers / Q000097:blood; D001809:Blood Viscosity / Q000502:physiology; D004903:Erythrocyte Aggregation / Q000502:physiology*; D004907:Erythrocyte Deformability / Q000502:physiology*; D004912:Erythrocytes / Q000473:pathology*; D006461:Hemolysis / Q000502:physiology*; D018056:Hemorheology / Q000502:physiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011317:Priapism / Q000097:blood* / Q000473:pathology; D011446:Prospective Studies; D012156:Reticulocytes / Q000473:pathology
chemical_list: D015415:Biomarkers