Lemonne Nathalie, Möckesch Berenike, Charlot Keyne, Garnier Yohann, Waltz Xavier, Lamarre Yann, Antoine-Jonville Sophie, Etienne-Julan Maryse, Hardy-Dessources Marie-Dominique, Romana Marc, Connes Philippe
Unité Transversale de la Drépanocytose, Hôpital Ricou, CHU de Pointe-á-Pitre, Guadeloupe, France.
Laboratoire ACTES, EA3596, Université des Antilles, Pointe-á-Pitre, Guadeloupe, France.
Clin Hemorheol Microcirc. 2017;67(2):141-148. doi: 10.3233/CH-170280.
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.
本研究的目的是测试羟基脲（HU）疗法对成年镰状细胞贫血（SCA）患者临床、血液学和血液流变学参数的影响。在28例SCA患者接受HU治疗前（即基线）以及治疗6个月、12个月和24个月时测量血液学和血液流变学参数。通过在30 Pa下的激光衍射法测定红细胞变形性。采用光背散射法研究红细胞聚集特性。用锥板粘度计在225 s-1下测量血液粘度。与基线相比，HU治疗1年和2年时血管闭塞性危象和急性胸部综合征的发生率较低。治疗2年后，腿部溃疡患者的比例有下降趋势。HU治疗可改善血红蛋白氧饱和度。HU治疗导致血小板和白细胞计数降低，胎儿血红蛋白水平和平均细胞体积升高。虽然在HU治疗下血红蛋白浓度增加，但在整个研究过程中血液粘度保持不变。HU治疗6个月时红细胞变形性高于基线值，并持续上升直至随访期结束。总之，红细胞变形性的改善可能抵消了血红蛋白增加对血液粘度的影响，并有助于改善患者的临床状况。
