Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis
Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis
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DOI：
10.1182/blood-2012-04-424184
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发表时间：
2012-10-11
期刊：
影响因子：
20.3
通讯作者：
Galacteros, Frederic
中科院分区：
文献类型：
--
作者：
Bartolucci, Pablo;Brugnara, Carlo;Galacteros, Frederic
致密脱水红细胞 (DRBC) 是镰状细胞病 (SCD) 的一个特征。 DRBCs 在 SCD 急性和慢性器官损伤的病理生理学中发挥着重要作用，因为它们的血红蛋白 S 浓度较高，因此更容易发生聚合和镰状化。在一大群纯合子患者中研究了红细胞密度（用邻苯二甲酸盐密度分布图方法评估）与一些血液学、生化、遗传参数和临床表现之间的关系。在经历过皮肤溃疡、阴茎异常勃起或肾功能障碍的患者中，DRBC 的百分比显着更高。 α-地中海贫血缺失的存在与较少的 DRBC 相关。多变量分析模型显示 DRBC 与乳酸脱氢酶和胆红素等溶血参数呈正相关，与胎儿血红蛋白呈负相关。羟基脲（羟基脲）治疗 6 个月后，DRBC 的百分比下降了 34%。因此，DRBC 与特定的临床表现和生物标志物相关，并且可能是对 SCD 患者的生物学和临床评估的有用补充，因为它们可以在血细胞比容管中轻松测量。 （血。2012；120（15）：3136-3141）
Dense, dehydrated red blood cells (DRBCs) are a characteristic feature of sickle-cell disease (SCD). DRBCs play a role in the pathophysiology of SCD acute and chronic organ damage because of heightened tendency to undergo polymerization and sickling because of their higher hemoglobin S concentration. Relations between red cell density (assessed with phthalate density-distribution profile method) and several hematologic, biochemical, genetic parameters, and clinical manifestations were studied in a large cohort of homozygous patients. The percentage of DRBCs was significantly higher in patients who experienced skin ulcers, priapism, or renal dysfunction. Presence of alpha-thalassemia deletions was associated with fewer DRBCs. A multivariable analysis model showed DRBCs to be positively associated with hemolytic parameters such as lactate dehydrogenase and bilirubin and negatively with fetal hemoglobin. The percentage of DRBCs decreased by 34% at 6 months of hydroxycarbamide (xydroxyurea) therapy. Thus, DRBCs are associated with specific clinical manifestations and biologic markers and may be a useful addition to the biologic and clinical evaluation of patients with SCD, because they can easily be measured in a hematocrit tube. (Blood. 2012; 120(15): 3136-3141)