阵发性睡眠性血红蛋白尿症血细胞免疫表型的研究

Immunophenotyping study on the blood cells of the patients with paroxysmal nocturnal hemoglobinuria

目的：探讨血细胞膜表面糖基磷脂酰肌醇（ＧＰＩ）锚蛋白分子表达异常在诊断阵发性睡眠性血红蛋白尿症（ＰＮＨ）时的意义。方法：应用一组单克隆抗体，采用免疫荧光法检测２２例ＰＮＨ患者粒细胞、红细胞、淋巴细胞的免疫表型。结果：发现ＰＮＨ患者粒细胞和红细胞膜表面与ＧＰＩ锚接蛋白有关的抗原ＣＤ５５和ＣＤ５９表达异常，而且较常规的溶血检查敏感。结论：检测血细胞膜表面ＧＰＩ锚蛋白分子表达可为确定或排除ＰＮＨ的诊断提供敏感、特异的证据。

Objective:To detect the expression of glycosyl phosphatidyl inositol (GPI) anchored protein on the blood cell membrane and its implication in the diagnosis of paroxysmal nocturnal hemoglobinuria(PNH).Methods:The GPI anchored protein was detected by indirect immunofluorescence method with a panel of monoclonal antibodies(CD 55 ,CD 59 ,CD 16 ,CD 58 ,HIR 2, CD 15 ,CD 2) in 22 PNH patients.Results:The abnormal granulocytes and erythrocytes with deficient GPI anchored proteins were identified in all PNH patients whether the routine hemolysis tests for PNH,including Ham test and mCLST, were positive or negative .However,lymphocytes with PNH defect were found in only 14 patients .Conclusion:GPI anchored protein assay provided sensitive and specific method for the diagnosis of PNH.