摘要目的:探讨系统性免疫球蛋白轻链淀粉样变性（AL）初治患者的外周血免疫细胞表型特征及其与临床指标的相关性。方法:采用流式细胞仪多参数免疫荧光分析技术，对36例AL初诊患者和28名健康供者的外周血单个核细胞的表面抗原CD3、CD56、CD4、CD8、CD25、CD45RA、CD28、CD57及核内抗原FOXP3进行检测和比较。根据梅奥2012分期对AL患者进行分期，比较Ⅰ~Ⅱ、Ⅲ~Ⅳ期患者的免疫细胞表型差异。分析λ轻链型AL患者T细胞亚群比例与多项临床指标的相关性。结果:AL患者的外周血T（CD3 +CD56 -）和NKT（CD3 +CD56 +）细胞比例，T细胞中的CD4 +CD8 -、CD4 -CD8 +、Treg（CD4 +CD25 +FOXP3 +）细胞比例与健康供者相比差异无统计学意义（ P>0.05）。AL患者的CD4 -CD8 +细胞中，CD57 +细胞的比例较健康供者显著降低（ P<0.05），但CD45RA +和CD28 +细胞的比例在AL和健康供者间差异无统计学意义。Ⅰ~Ⅱ期和Ⅲ~Ⅳ期AL患者T细胞及其亚群的比例差异无统计学意义（ P>0.05）。在λ轻链型AL患者中，外周血CD4 -CD8 +细胞的比例与24 h尿蛋白和血肌酐呈正相关（ P<0.05），与eGFR呈负相关（ P<0.05），与其他临床指标无显著相关性。与此相反，CD4 +CD8 -细胞的比例与eGFR呈正相关，而与24 h尿蛋白和血肌酐呈负相关（ P<0.05）。 结论:AL患者外周血的T细胞亚群与健康供者相比差异无统计学意义，但CD8 + T细胞的比例与肾脏损伤程度呈正相关，提示CD8 + T细胞的比例在评估AL患者肾脏预后中具有一定的价值。

abstractsObjective:To investigate the characteristics of T cell immunophenotype and its relationship with clinical manifestation in patients with systemic light chain amyloidosis (AL) .Methods:The peripheral blood mononuclear cells from 36 patients with AL were collected and analyzed by multicolor flow cytometry, and the expression of surface antigen CD3, CD56, CD4, CD8, CD25, CD45RA, CD28, CD57 and nuclear antigen FOXP3 were examined. Samples from 28 age-matched healthy donors (HD) were also examined. Patients were divided by Mayo 2012 staging system and the difference between immunophenotype of Ⅰ-Ⅱ and Ⅲ-Ⅳ stage patients were analyzed. The correlations between the proportion of T-cell subpopulation and clinical manifestations in λ light chain type AL patients were analyzed.Results:The differences in the peripheral total T cells and T cell subsets, including CD4 +, CD8 +, regulatory T cells, and natural killer T cells were not significantly between AL and HD. The ratio of CD57 + cells in CD8 + T cells was lower in AL than in HD, and there was no significantly difference in the rate of CD45RA + and CD28 +cells between these two groups. No differences were found in the ratio of total T cells or T cell subsets between stages Ⅰ-Ⅱ and Ⅲ-Ⅳaccording to the standard of Mayo 2012. Within λ light chain type AL patients, peripheral CD8 + T cell ratio was positively correlated with 24-hour urine protein and creatinine level and negatively correlated with estimated glomerular filtration rate (eGFR) . Conclusion:The overall T cell distribution in the periphery is not significantly different between AL patients and age-matched healthy donors. However, the percentages of CD8 + T cells are positively correlated with renal injury, indicating the importance of CD8 + T cell subset in the prognostic evaluation of renal involvement.