Galey W R, Evan A P, Van Nice P S, Dail W G, Wimer B M, Cooper R A
Vox Sang. 1978;34(3):152-61. doi: 10.1111/j.1423-0410.1978.tb02458.x.
The McLeod phenotype is a rare condition characterized by deficiencies in the Kell blood group antigens of erythrocytes. The present study has defined some of the morphological and physiological characteristics of these red cells. Scanning electron microscopy reveals that approximately 27% of McLeod cells are morphologically abnormal. These aberrant forms resemble the acanthocytes of abetalipoproteinemia. Incubation experiments indicate that the morphology of McLeod erythrocytes is not due to a plasma factor. Biochemical studies reveal that the lipid composition and microviscosity of these erythrocytes are normal. In addition, isotopic tracer experiments indicate that electrolyte transport is also within the normal range. However, osmotic water permeability is approximately 30% below normal. The absence of a lipid abnormality and other experimental data suggest that the abnormal morphology and reduced H2O permeability of the McLeod erythrocyte may be due to defective membrane protein.
麦克劳德表型是一种罕见病症,其特征是红细胞的凯尔血型抗原缺乏。本研究确定了这些红细胞的一些形态学和生理学特征。扫描电子显微镜显示,约27%的麦克劳德细胞在形态上异常。这些异常形态类似于无β脂蛋白血症的棘红细胞。孵育实验表明,麦克劳德红细胞的形态并非由血浆因子所致。生化研究显示,这些红细胞的脂质组成和微粘度正常。此外,同位素示踪实验表明电解质转运也在正常范围内。然而,渗透水通透性比正常水平低约30%。脂质异常的不存在及其他实验数据表明,麦克劳德红细胞异常的形态和降低的水通透性可能是由于膜蛋白缺陷所致。