白血病和骨髓增生异常综合征患者红细胞酶及同工酶谱的改变

目的：调查白血病和骨髓增生异常综合征（ＭＤＳ）患者红细胞酶和同工酶谱改变的患病率，并研究其临床意义。方法：采用ＩＣＳＨ介绍的方法及ＰＡＧＥ电泳测定红细胞酶活性及ＰＫ和ＡＤＡ同工酶，采用ＦＰＬＣ系统纯化ＡＤＡ同工酶，分析酶蛋白的生化性质。结果：患病率：在２２９例白血病和ＭＤＳ患者中，Ｇ６ＰＤ缺乏为４３．１％，ＰＫ缺乏为２７．８％。ＭＤＳ患者中的酶缺乏患病率：ＰＫ缺乏为５０．０％，Ｇ６ＰＤ缺乏为４８．０％，ＰＮＰ缺乏为３９．１％；酶过多患病率：ＡＤＡ过多为６９．６％，ＥＮＯＬ过多为４０．０％，ＡＬＤ过多为３０．４％。慢性粒细胞白血病（ＣＭＬ）的ＰＫ活性增高，而ＰＫ同工酶型均正常。急性白血病患者采用抗肿瘤抗生素后Ｇ６ＰＤ和６ＰＧＤ活性减低（Ｐ＜０．０５）。ＭＤＳ红细胞酶缺陷显著多于ＡＤＡ２同工酶表达见于ＡＭＬ（Ｍ４和Ｍ５）的单个核细胞及慢性粒－单核细胞白血病（ＣＭＭＬ）的红细胞，但ＡＬＬ不表达（Ｐ＜０．００１）。ＡＤＡ同工酶纯化分析示正常结构。结论：红细胞酶的检测可作为ＣＭＬ的预后参数，作为急性白血病抗肿瘤药物耐药预示指标，有助于ＭＤＳ－ＲＡ和再障、ＣＭＬ与ＣＭＭＬ的鉴别诊断。

Study on red cell enzymes and isoenzymes in patients with leukemia and myelodysplastic syndromes

Objective:To define the prevalence of acquired red cell enzymopathy in leukemia and MDS patients and explore its clinical significance.Methods:Red cell enzymes (G6PD,6PGD,PK,ENOL,ADA,PNP,ALD) activities and PK,ADA isoenzymes were assayed by the methods recommended by ICSH and PAGE electrophoresis.Results:The prevalence rate of G6PD deficiency was 43.1%,and of PK deficiency was 27.8%,in leukemia and MDS patients.The prevalence rates of PK,G6PD and PNP deficiency in MDS patients were 50%,48% and 39.1%,respectively,while of ADA,ENOL and ALD over production in MDS patients were 69.6%,40% and 30.4%,respectively.PK activity was increased in CML, while the PK isoenzyme pattern was normal.Administration of antitumor antibiotics to AL decreased G6PD and 6PGD activities (P<0.05).ADA2 was expressed in MNC of AML(M 4 and M 5) and in RBC of CMML,but not expressed in ALL(P<0.001).Conclusion:Determination of enzymes may be used as prognostic parameters in CML,a predictor of antitumor drug resistance in acute leukemia,and a differential parameter between MDS RA and aplastic anemia and between CML and CMML.