Affiliations: [a] Unité Transversale de la Drépanocytose, Hôpital Ricou, CHU de Pointe-á-Pitre, Guadeloupe, France
| [b] Laboratoire ACTES, EA3596, Université des Antilles, Pointe-á-Pitre, Guadeloupe, France
| [c] Inserm UMR 1134, Hôpital Ricou, CHU de Pointe-á-Pitre, Guadeloupe, France
| [d] Laboratoire d’Excellence GR-Ex « The red cell: from genesis to death », PRES Sorbonne Paris Cité, Paris, France
| [e]
Armed Forces Biomedical Research Institute, Brétigny-sur-Orge, France
| [f] Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM), EA7424, Equipe Biologie Vasculaire et du Globule Rouge, Université Claude Bernard Lyon 1, COMUE Lyon, Villeurbanne, France
| [g] Institut Universitaire de France, Paris, France
Correspondence:
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Corresponding author: Philippe Connes, Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM), EA7424, Equipe Biologie Vasculaire et du Globule Rouge, Université Claude Bernard Lyon 1, France. E-mails: [email protected]; [email protected].
Abstract: The aim of the present study was to test the effects of hydroxyurea (HU) therapy on clinical, hematological and hemorheological parameters in adult patients with sickle cell anemia (SCA). Hematological and hemorheological parameters were measured in 28 SCA patients before HU therapy (i.e., baseline) and at 6, 12 and 24 months of treatment. RBC deformability was determined by ektacytometry at 30 Pa. RBC aggregation properties were investigated by light-backscatter method. Blood viscosity was measured at 225 s–1 by a cone-plate viscometer. The rates of vaso-occlusive crises and acute chest syndrome were lower at 1 and 2 years of HU therapy compared to baseline. The proportion of patients with leg ulcers tended to decrease after 2 years of treatment. Hemoglobin oxygen saturation improved with HU therapy. HU therapy induced a decrease of platelet and white blood cell counts and a rise in fetal hemoglobin level and mean cell volume. While hemoglobin concentrations increased under HU, blood viscosity remained unchanged all along the study. RBC deformability increased over baseline values at 6 months of HU therapy and continued to rise until the end of the follow-up period. In conclusion, the improvement in RBC deformability probably compensates the increase of hemoglobin on blood viscosity and participates to the improvement of the clinical status of patients.
