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Not all red cells sickle the same: Contributions of the reticulocyte to disease pathology in sickle cell anemia.
Blood Reviews ( IF 6.9 ) Pub Date : 2019-11-05 , DOI: 10.1016/j.blre.2019.100637
Marcus A Carden 1 , Ross M Fasano 2 , Emily Riehm Meier 3
Affiliation  

Sickle cell anemia (SCA) is associated with morbidity and early death. While the switch from fetal to sickle hemoglobin during the first months of life results in hemolytic anemia with reticulocytosis, the role of the reticulocyte in the pathophysiology and prognosis of SCA is not well-defined. Reticulocytes have unique cytoskeletal and membrane components that allow them to be distinguished from mature sickle erythrocytes in the circulation. Reticulocytes in patients with SCA are less dense than more mature and ‘sickled’ erythrocytes, and have increased adhesive properties. The circulating reticulocyte number in peripheral blood may assist in predicting disease severity in SCA; characterization of patient-specific reticulocyte properties during infancy and childhood may assist in predicting therapeutic response to therapies. Here, we review the biological and clinical data regarding reticulocytes and their potential impact on SCA pathophysiology and disease severity.



中文翻译:

并非所有的红细胞都具有相同的镰状:镰状细胞性贫血中网状细胞对疾病病理的贡献。

镰状细胞性贫血(SCA)与发病率和早期死亡相关。虽然在生命的最初几个月中从胎儿血红蛋白转换为镰状血红蛋白会导致溶血性贫血伴网织红细胞增多,但网织红细胞在SCA的病理生理和预后中的作用尚不明确。网织红细胞具有独特的细胞骨架和膜成分,可使其与循环中成熟的镰状红细胞区分开来。患有SCA的患者的网状细胞密度不如较成熟和“带病”的红细胞密度高,并且具有增加的黏附特性。外周血中循环网织红细胞数量可能有助于预测SCA中的疾病严重程度;婴儿期和儿童期患者特异性网织红细胞特性的表征可能有助于预测对疗法的治疗反应。这里,

更新日期:2019-11-05
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