阵发性睡眠性血红蛋白尿症患者外周血T淋巴细胞亚群及活化分子表达的研究

目的　探讨阵发性睡眠性血红蛋白尿症 (PNH)患者T淋巴细胞的功能及活化状态与PNH临床的关系。方法　应用流式细胞术及免疫磁珠分选术 ,测定了 18例PNH患者外周血单个核细胞 (PBMNC)及CD59+ 和CD59-PBMNC中CD3 + 、CD4+ 及CD8+ 细胞表型 ,并测定了新诊断的未经治疗的 6例PNH患者外周血中NK细胞及CD4+ CD2 8+ /CD4+ 、CD8+ CD2 8+ /CD8+ 、CD4+ HLR DR+ /CD4+ 、CD8+HLR DR+ /CD8+ 及CD8+ CD3 8+ /CD8+ 淋巴细胞表面分子表达比值。结果　PNH患者PBMNC中CD3 +CD8+ /CD3 + CD4+ 细胞比值增加 ,为 1.2 2± 0 .5 1,对照组为 0 .86± 0 .2 7,两者比较 ,差异有显著性 (P <0 .0 5 )。分选后PNH患者CD59-PBMNC中CD3 + CD8+ /CD3 + CD4+ 细胞比值增加 ,为 2 .31± 1.5 6 ,CD59+PBMNC为 0 .6 2± 0 .2 7,两者比较 ,差异有统计学意义 (P <0 .0 1)。CD3 + CD8+ /CD3 + CD4+ 细胞比值与骨髓衰竭 (BMF)的级差相关分析呈正相关。PNH患者CD4+ CD2 8+ /CD4+ 细胞比值明显减少 ,为 0 .5 2±0 .11(对照为 1.0 0± 0 .0 6 ) ,而CD8+ HLR DR+ /CD8+ 增加 ,为 0 .4 5± 0 .2 6 (对照为 0 .10± 0 .0 6 )。结论　PNH患者CD3 + CD8+ /CD3 + CD4+ 细胞比值增加 ,病变表型细胞更易发生在CD8+ 细胞群中。CD4+ 细胞中CD2 8+ 辅助刺激因

Study of lymphocyte subsets and its activated-molecules in patients with paroxysmal nocturnal hemoglobinuria

OBJECTIVE: To investigate the relationship between subsets of lymphocytes and between its activated status and the clinical manifestations in patients with PNH, and to unfold immunological mechanism in the pathogenesis of PNH. METHODS: The peripheral blood mononuclear cells (PBMNC) from 18 PNH patients and 20 controls were separated into two subpopulations using anti-CD(59) monoclonal antibody combined with goat-anti-mouse IgG immunomagnetic beads. CD(3)(+), CD(4)(+) and CD(8)(+) lymphocyte subsets were detected by flow cytometry. In 6 newly diagnosed patients, phenotypes associated with T cell activation such as CD(28)(+)/CD(4)(+) or CD(8)(+) cells, CD(8)(+) CD(38)(+) cells, and HLA-DR(+)/CD(4)(+) or CD(8)(+), and NK (CD(3)(-) CD(16)(+)) cells were detected in the peripheral blood. RESULT: Patients with PNH showed significantly increased CD(3)(+) CD(8)(+)/CD(3)(+) CD(4)(+) ratio as compared with controls (1.22 +/- 0.51 vs 0.86 +/- 0.27, P < 0.05), and the CD(3)(+) CD(8)(+)/CD(3)(+) CD(4)(+) ratio in CD(59)(-) PBMC was higher than that in CD(59)(+) PBMC (2.31 +/- 1.56 vs 0.62 +/- 0.27, P < 0.05). The ratios of CD(4)(+) CD(28)(+)/CD(4)(+) markedly decreased and CD(8)(+)HLA-DR(+)/CD(8)(+) increased. CONCLUSION: Patients with PNH appear to have abnormalities in their lymphocytes. Increased ratios of CD(3)(+) CD(8)(+)/CD(3)(+) CD(4)(+) and HLA-DR(+) CD(8)(+)/CD(8)(+) lymphocytes as well as declined ratio of CD(4)(+) CD(28)(+)/CD(4)(+) lymphocytes might be involved in the pathogenesis of PNH.