骨髓增生异常综合征红细胞酶谱测定临床应用研究

Clinical Use of Determination of Erythrocyte Enzyme Activity in Myelodysplastic Syndromes

本文比较了２３例ＭＤＳ和９例ＡＡ患者４种红细胞酶的活性，除醛缩酶（ＡＬＤ）外，丙酮酸激酶（ＰＫ）、嘌呤核苷磷酸化酶（ＰＮＰ）、腺苷脱氨酶（ＡＤＡ）在ＡＡ患者均接近正常，和ＭＤＳ差异显著，尤其是ＰＫ和ＰＮＰ差异显著。据此联合检测红细胞４种酶活性，建立了ＭＤＳ和ＡＡ的判别函数式：ＰＭＤＳ＝－５．０４１０７＋０．２５６７４ＰＫ＋１．６７６５１ＡＬＤ＋０．０３４８１ＰＮＰ＋０．２５０２４ＡＤＡ，ＰＡＡ＝－７．９００３８＋０．３８４６ＰＫ＋１．４９６２１ＡＬＤ＋０．０８０５ＰＮＰ＋０．１３２３７ＡＤＡ。内部回代结果，判别敏感度ＭＤＳ为８２．６１％，ＡＡ为７７．７８％，特异度ＭＤＳ可达９０．４８％，ＡＡ仅为６３．６４％。结果表明，该判别函数式可反映红系病态造血用于临床作为ＭＤＳ诊断的辅助指标。

The activities of four erythrocyte enzymes, pyruvate kinase(PK), aldolase(ALD), purine nucleoside phosphorylase(PNP), and adenosine deaminase(ADA) were determined among 23 patients with myelodysplastic syndromes(MDS) and 9 cases of aplastic anemia(AA). The comparison study showed that erythrocyte enzyme activities, except ALD, in patient with AA were close to the normal range and were significantly different from that of MDS patients, expecially for PK and PNP activities. A discriminant function for distinguishing MDS from AA was established as follow: P(MDS)=-5.04107+0.25674PK+1.67651ALD+0. 03481PNP+0.25024ADA. P(AA)=-7.90038+0.3846PK+1.49621ALD+0.0805PNP+0.13237ADA. For the diagnosis of MDS, the sensitivity was 82.61%, the specificity was 90.48%, This discriminant function could reflect the dyshematopoiesis and be used as the supplementary diagnostic index of MDS.