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溶血性贫血和骨髓增殖性疾病中的红细胞膜磷脂酰肌醇激酶活性

Red cell membrane phosphatidylinositol kinase activity in hemolytic anemias and myeloproliferative diseases.

作者信息

Boivin P, Galand C

机构信息

INSERM U 160, Association Claude Bernard, Hôpital Beaujon, Clichy, France.

出版信息

Clin Chim Acta. 1989 Jun 30;182(2):165-72. doi: 10.1016/0009-8981(89)90075-2.

DOI:10.1016/0009-8981(89)90075-2
PMID:2550165
Abstract

Phosphatidylinositol kinase activity was determined in red cell membranes from 85 healthy individuals, 20 patients with hereditary hemolytic anemia and 24 patients with myeloproliferative disorder. Increased activity was found in all ten cases of sickle red disease and seven among ten cases of other hereditary hemolytic anemias. These increases had no correlation with the reticulocyte count nor with the red cell shape. An unexpected decreased activity was found in several cases of myeloproliferative disorders, especially in polycythemia vera, with a negative correlation with the reticulocyte count. The mechanism(s) and significance of the phosphatidylinositol kinase abnormalities in these different groups of diseases remain to determine.

摘要

在85名健康个体、20名遗传性溶血性贫血患者和24名骨髓增殖性疾病患者的红细胞膜中测定了磷脂酰肌醇激酶活性。在所有10例镰状红细胞病患者以及10例其他遗传性溶血性贫血患者中的7例中发现活性增加。这些增加与网织红细胞计数以及红细胞形态均无相关性。在几例骨髓增殖性疾病患者中,尤其是真性红细胞增多症患者中发现了意外的活性降低,且与网织红细胞计数呈负相关。这些不同疾病组中磷脂酰肌醇激酶异常的机制和意义仍有待确定。

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