Increased surface receptor Fas (CD95) levels on CD4+lymphocytes in patients with primary intestinal lymphangiectasia
Increased surface receptor Fas (CD95) levels on CD4+lymphocytes in patients with primary intestinal lymphangiectasia
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DOI：
10.1080/00365520802321220
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发表时间：
2009-01-01
影响因子：
1.9
通讯作者：
Carcelain, Guislaine
中科院分区：
文献类型：
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作者：
Vignes, Stephane;Carcelain, Guislaine
Objective.继发于原发性肠淋巴管扩张症（PIL）的渗出性肠病的特征是淋巴液渗漏进入肠道导致的淋巴细胞减少症、低丙种球蛋白血症和低白蛋白血症。本研究的目的是更好地描述PIL确认患者的淋巴细胞减少症。材料和方法。对9例患者（6例女性，3例男性，年龄18 - 72岁）的T细胞标志物和T细胞增殖/能力（分化、活化和死亡）进行了表型分析。结果与对照组（分别为858260/l和482164/l）相比，CD 4和CD 8亚群的平均计数显著降低（174123/l和13477/l）（p <0.0001）。观察到初始（CD 45 RA + CD 62 L+）CD 4 + T细胞显着消耗，平均表达率为74%，而对照组为451%（p0.0001）。通过表达晚期活化标志物HLA-DR的比例评估，CD 4+和CD 8 + T细胞平均亚群均活化，分别为187%和199%，而对照组分别为63%和106%（p <0.0001和p <0.01）。患者中CD 4 + T细胞上CD 95/Fas的平均表达显著高于对照组，分别为8316%和4513%（p <0.0001）。未观察到T细胞增殖/能力的重大异常。结论.我们的研究结果表明，PIL患者的T细胞丢失可能是由于多种机制，包括肠道淋巴细胞丢失和活化残留的T细胞，导致死亡。此外，这种损失不能通过T细胞胸腺产生的充分增加来补偿。
Objective. Exudative enteropathy secondary to primary intestinal lymphangiectasia (PIL) is characterized by lymphopenia, hypogammaglobulinemia and hypoalbuminemia resulting from leakage of lymph fluid into the intestinal tract. The objective of this study was to better characterize the lymphopenia of PIL-confirmed patients. Material and methods. T-cell markers and T-cell proliferation/capacities (differentiation, activation and death) were analyzed for phenotype in 9 patients (6 F, 3 M, aged from 18 to 72 years). Results. Mean counts of CD4 and CD8 subsets were significantly decreased, 174123/l and 13477/l compared with controls, 858260/l and 482164/l, respectively (p0.0001). Significant depletion of naive (CD45RA+ CD62L+) CD4+ T cells was noted, with a mean expression of 74% compared with controls, 451% (p0.0001). Both CD4+ and CD8+ T-cell mean subsets were activated as assessed by their proportion expressing the late activation markers HLA-DR, 187% and 199% compared with controls, 63% and 106%, respectively (p0.0001 and p0.01). The mean expression of CD95/Fas on CD4+ T cells was significantly higher in patients than in controls, 8316% versus 4513% (p0.0001). No major abnormality of T-cell proliferation/capacities was observed. Conclusions. Our results suggest that the T-cell loss in PIL patients is probably due to various mechanisms including enteric lymphocytes loss and activation of residual T cells, leading to death. Moreover, this loss is not compensated by a sufficient increase in T-cell thymic production.