GPI锚定蛋白检测诊断阵发性睡眠性血红蛋白尿症的临床价值

Clinical value of glycosyl-phosphatidyl inositol anchored protein detection on diagnosis of paroxysmal nocturnal hemoglobinuria

目的:探讨阵发性睡眠性血红蛋白尿症(PNH)患者不同外周血细胞GPI锚定蛋白的表达情况,为PNH的诊断和鉴别诊断提供依据。方法:采用流式细胞术检测32例PNH患者外周血各类细胞膜上CD55、CD59、CD16及CD14的表达百分率。结果:PNH患者外周血红细胞、粒细胞膜上CD55、CD59表达均明显减少,与正常对照组比较差异有显著性(P<0.001),且粒细胞上CD55、CD59表达率低于红细胞(P<0.05);血小板CD55、CD59表达率也较对照组减少(P<0.05);有27例PNH患者的淋巴细胞CD55、CD59表达降低,其缺失率达30%,低于粒细胞和红细胞;CD16在粒细胞上表达量差异很大(34%～83%),与对照组比较差异有显著性(P<0.001);在单核细胞上可见CD14表达缺失的PNH克隆,CD14表达比例明显低于正常对照组(P<0.001)。结论:检测外周各系血细胞CD55和CD59可以使典型PNH的诊断更加可靠,使部分缺乏典型临床表现的PNH明确诊断。检测CD16和CD14对排除PNH具有重要意义。

Objective To investigate the expression of glycosyl-phosphatidyl inositol(GPI) anchored protein on peripheral blood cells from patients with paroxysmal nocturnal hemoglobinuria (PNH) and provide a basis for diagnosis and differential diagnosis of PNH.Methods The percentages of CD55,CD59,CD16 and CD14 expression on the peripheral blood cells from 32 patients with PNH were determined by flow cytometry. Results The expression levels of CD55 and CD59 were decreased significantly on the erythrocytes and granuloc...