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白血病前期伴溶血的获得性丙酮酸激酶缺乏症

Acquired pyruvate kinase deficiency with hemolysis in preleukemia.

作者信息

Helmstädter V, Arnold H, Blume K G, Uhl N, Hunstein W

出版信息

Acta Haematol. 1977;57(6):339-43. doi: 10.1159/000207900.

Abstract

Acquired erythrocyte pyruvate kinase deficiency may appear as a symptom secondary to various hematologic disorders, e.g. acute leukemia, sideroblastic anemia, polycythemia vera. The case of a 68-year-old patient with PK deficiency (1.75 U/g Hb) and severe hemolytic anemia is presented, who 1 year later showed acute myeloid leukemia. It is considered that a dialysable inhibiting factor may play a pathogenetic role in this enzyme change since enzyme activity was raised by dialysis. A survey of the literature is presented.

摘要

获得性红细胞丙酮酸激酶缺乏症可能表现为继发于各种血液系统疾病的症状,例如急性白血病、铁粒幼细胞贫血、真性红细胞增多症。本文报道了一例68岁的丙酮酸激酶缺乏症(1.75 U/g血红蛋白)和严重溶血性贫血患者,该患者1年后出现急性髓系白血病。鉴于透析可提高酶活性,推测一种可透析的抑制因子可能在这种酶变化中起致病作用。本文还对相关文献进行了综述。

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