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己糖胺酶同工酶在儿童白血病中的表达

Expression of hexosaminidase isoenzymes in childhood leukemia.

作者信息

Ellis R B, Rapson N T, Patrick A D, Greaves M F

出版信息

N Engl J Med. 1978 Mar 2;298(9):476-80. doi: 10.1056/NEJM197803022980902.

Abstract

Because differences in hexosaminidase isoenzyme profiles of granulocytes and lymphoyctes suggested that such profiles might help to distinguish between various types of leukemia, was examined leukocyte extracts from 55 untreated children and 12 controls by automated anion-exchange chromatography. In 23 of 27 cases of the common form of childhood acute lymphoblastic leukemia, the activity of hexosaminidase component I was greatly increased; the activity ratio of hexosaminidase I to hexosaminidase A was greater than 0.5 in 18 of these cases, whereas for other types of leukemia and for all normal cells tested, the ratio was less than 0.2. A raised hexosaminidase I was shown to be associated with leukemic cells by the finding of normal isoenzyme profiles of bone-marrow cells from 17 patients in remission who had such an increase either at diagnosis or in relapse. Hexosaminidase isoenzyme analysis had diagnostic value and may provide a new marker for study of leukocyte differentiation.

摘要

由于粒细胞和淋巴细胞的己糖胺酶同工酶谱存在差异,提示这种谱型可能有助于区分不同类型的白血病,因此采用自动阴离子交换色谱法对55例未经治疗的儿童和12例对照的白细胞提取物进行了检测。在27例儿童急性淋巴细胞白血病常见类型的病例中,有23例己糖胺酶成分I的活性大幅增加;其中18例己糖胺酶I与己糖胺酶A的活性比大于0.5,而对于其他类型的白血病以及所有检测的正常细胞,该比值均小于0.2。通过对17例缓解期患者骨髓细胞的同工酶谱进行检测,发现这些患者在诊断或复发时己糖胺酶I升高,且骨髓细胞同工酶谱正常,表明升高的己糖胺酶I与白血病细胞有关。己糖胺酶同工酶分析具有诊断价值,可能为白细胞分化研究提供一种新的标志物。

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