补体调节蛋白在三种干细胞缺陷性贫血中的表达及其意义的研究  

Studies on the expression of complement regulatory proteins in three kinds of stem cell defect anemias and its clinical implications

作　　者：徐从高[1] 张锑[1] 赵睿 张茂宏[1] 

机构地区：[1]山东医科大学附属医院血液学研究室,济南250012

出　　处：《中华血液学杂志》1999年第10期511-514,共4页Chinese Journal of Hematology

摘　　要：目的　观察糖化磷脂酰肌醇（ＧＰＩ） 连接的补体调节蛋白（ＣＲＰ） ———ＣＤ５５ 和ＣＤ５９ 在阵发性睡眠性血红蛋白尿症（ＰＮＨ） 、再生障碍性贫血（ 再障） 和骨髓增生异常综合征（ＭＤＳ） 患者的外周血血细胞表达的变化并分析其在上述疾病中的临床意义。方法　采用荧光标记抗ＣＤ５５ 和ＣＤ５９ 单克隆抗体，流式细胞术测定３０ 名正常人，２１ 例ＰＮＨ患者，３１ 例再障患者及２１ 例ＭＤＳ患者外周血中性粒细胞、淋巴细胞和红细胞膜抗原的表达。结果　ＰＮＨ 患者三种血细胞的ＣＤ５９ 表达均明显降低，而ＣＤ５５ 的降低只见于中性粒细胞和红细胞；ＰＮＨ患者的ＣＤ５９－ 中性粒细胞比例均高于１０％ ，红细胞ＣＤ５９ 表达情况与疾病的严重程度相关。再障患者中性粒细胞表达ＣＤ５５ 和ＣＤ５９ 亦较对照显著降低，但程度比ＰＮＨ 患者为轻。ＭＤＳ患者血细胞ＣＤ５５ 和ＣＤ５９的表达无显著变化。结论　测定ＣＲＰ在ＰＮＨ诊断和鉴别诊断中具有重要价值；就诊断敏感性和特异性而言，ＣＤ５９优于ＣＤ５５ ，中性粒细胞优于淋巴细胞和红细胞。再障患者血细胞ＣＲＰ的降低表明其与ＰＮＨ 关系密切，但欲通过测定ＣＲＰ鉴别两者，则须进一步确定其判别界限。有时再障与低增生性ＭＤＳ?Objective To investigate the expression of membrane associated complement regulatory proteins(CRP)——CD 55 and CD 59 on the peripheral blood cells in patients with paroxysmal nocturnal hemoglobinuria(PNH),aplastic anemia(AA) and myelodysplastic syndrome (MDS) and its clinical implications.Methods Expression of CD 55 and CD 59 on neutrophils,lymphocytes and erythrocytes was analyzed with flow cytometry, using FITC and PE labeled anti CD 55 and CD 59 monoclonal antibodies. Results CD 55 and CD 59 expression on neutrophils was significantly decreased in 21 PNH patients (including 2 cases of AA PNH syndrome). CD 59 - cells in neutrophils were constantly in excess of 10% in all of the PNH patients .Reduction in CD 59 expression on erythrocytes was related to the severity of the disease.CD 55 - neutrophils were also significantly increased in PNH patients but with a greater variation.Expressions of CD 55 and CD 59 on neutrophils were remarkably decreased in 31 AA patients but to a less extent as compared to PNH.No significant difference in the expressions of CD 55 and CD 59 was found in all the 21 MDS patients.Conclusion Expression of CRP is reduced in PNH and its related disorder AA but remains unchanged in MDS.Flow cytometric analysis of complement regulatory proteins is of value in diagnosis and differential diagnosis of PNH,AA and MDS.In terms of diagnostic sensitivity and specificity,CD 59 is superior to CD 55 ,and neutrophil is superior to lymphocyte and erythrocyte.

关 键 词：血红蛋白尿 再生障碍性贫血 MDS 补体调结蛋白 

分 类 号：R556.64[医药卫生—血液循环系统疾病] R556.5[医药卫生—内科学]