Deformability of erythrocytes from four patients with different types of glucosephosphate isomerase (D-glucose-6-phosphate ketoisomerase, GPI) deficiency has been determined by cell filtration. Young as well as whole erythrocyte populations had a markedly increased rigidity and an abnormally strong attachment of haemoglobin to the inner surface of isolated membranes. Acidic environment may enhance membran rigidity in vitro and also during passage of the erythrocytes through the spleen. The decrease of deformability at a pH of 6.8 was most pronounced in the splenectomized patients, and likewise in erythrocytes from the splenic artery, which were obtained from one patient during splenectomy. It is suggested that the metabolic environment of the spleen, with its low pH, impairs the deformability of GPI-deficient erythrocytes and predisposes them to splenic sequestration. The clinical improvement of all patients following splenectomy which is accompanied by an increase of the erythrocyte survival time and by unchanged reticulocyte counts, is in accordance with this view.

译文

:已通过细胞过滤确定了四名患有不同类型葡萄糖磷酸异构酶(D-葡萄糖-6-磷酸酮异构酶,GPI)缺乏症患者的红细胞变形能力。年轻的和整个红细胞群体的刚度显着增加,并且血红蛋白异常强烈地附着在离体膜的内表面上。酸性环境可以在体外以及在红细胞通过脾脏的过程中增强膜的刚性。在脾切除的患者中,pH值为6.8时变形能力的降低最为明显,同样,在脾切除术中从一名患者获得的脾动脉的红细胞中也是如此。提示脾脏的代谢环境具有较低的pH值,会损害GPI缺失型红细胞的变形能力,并使它们易于进行脾隔离。该观点认为,脾切除术后所有患者的临床改善均伴随着红细胞存活时间的增加和网织红细胞计数的不变。

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