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伴有葡萄糖-6-磷酸脱氢酶汉堡型的红细胞膜变形性

Membrane deformability of erythrocytes with glucose-6-phosphate dehydrogenase Hamburg.

作者信息

Tillmann W, Gahr M, Labitzke N, Schröter W

出版信息

Acta Haematol. 1977;57(3):162-7. doi: 10.1159/000207876.

Abstract

Deformability of erythrocytes of a patient with chronic nonspherocytic hemolytic anemia caused by a G-6-PD variant (G-6-PD Hamburg) in red cells was studied. The flow rate of erythrocytes from this patient through 5 mum polycarbonate sieves was higher than that of red cells from healthy adults. Even under oxidative stress in vitro, the deformability of erythrocytes of the patient was only slightly decreased. The residual hemoglobin content of red cell membranes (ghosts) from this patient was lower than that of ghosts from healthy adults and of ghosts prepared from comparable reticulocyte-rich blood withoug G-6-PD deficiency. In contrast to the low enzymatic activity of G-6-PD Hamburg in the hemolysate, a high activity of this enzyme could be demonstrated in the ghosts from the patient. In view of the flexibility of this patient's erythrocytes splenectomy is not to be recommended.

摘要

对一名由红细胞中G-6-PD变体(G-6-PD汉堡型)引起的慢性非球形细胞溶血性贫血患者的红细胞变形性进行了研究。该患者的红细胞通过5微米聚碳酸酯筛网的流速高于健康成年人的红细胞。即使在体外氧化应激下,该患者红细胞的变形性也仅略有下降。该患者红细胞膜(血影)的残余血红蛋白含量低于健康成年人血影以及由不含G-6-PD缺乏的可比富含网织红细胞的血液制备的血影。与溶血产物中G-6-PD汉堡型的低酶活性相反,在该患者的血影中可证明该酶具有高活性。鉴于该患者红细胞的柔韧性,不建议进行脾切除术。

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