Density, heterogeneity and deformability of red cells as markers of clinical severity in hereditary spherocytosis复制标题

红细胞密度、异质性和变形性作为遗传性球形红细胞增多症临床严重程度的标志物

Hereditary spherocytosis originates from defective anchoring of the cytoskeletal network to the transmembrane protein complexes of the red blood cell. Red cells in hereditary spherocytosis are characterized by membrane instability, reduced deformability and a large heterogeneity in disease severity among patients. To unravel this variability in disease severity, we analyzed blood samples from 21 spherocytosis patients with defects in ankyrin, band 3, α-spectrin and β-spectrin using red cell indices, eosin-5-maleimide binding, microscopy and the osmotic fragility test, Percoll density gradients, vesiculation and ektacytometry to assess cell membrane stability, cellular density and deformability. Reticulocyte counts, CD71 abundance, band 4.1 a:b ratio, and HbA1c were used as markers of red blood cell turnover. We observed that patients with moderate/severe spherocytosis have short-living erythrocytes of low density and abnormally high intercellular heterogeneity.

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