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小儿风湿性疾病中αβTCR双阴性T细胞的非特异性增加。

Nonspecific increase of αβTCR double-negative T cells in pediatric rheumatic diseases.

作者信息

Dossybayeva Kuanysh, Zhubanova Gulsamal, Mussayeva Assel, Mukusheva Zaure, Dildabayeva Aiken, Nauryzbayeva Galiya, Akhmaltdinova Lyudmila, Orumbayeva Ulbolsyn, Tanko Matthew, Poddighe Dimitri

机构信息

Department of Medicine, Nazarbayev University School of Medicine (NUSOM), 010000, Astana, Kazakhstan.

Program of Pediatric Rheumatology, Clinical Academic Department of Pediatrics, University Medical Center, Astana, Kazakhstan.

出版信息

World J Pediatr. 2024 Dec;20(12):1283-1292. doi: 10.1007/s12519-024-00854-7. Epub 2024 Nov 28.

Abstract

BACKGROUND

An increased number of double-negative T (DNT) cells expressing the αβ T cell receptor (αβDNT cells) is one of the diagnostic criteria for autoimmune lymphoproliferative syndrome (ALPS). Moreover, these cells are expanded in a widely used murine model for lupus. However, the homeostasis of αβDNT cells remains inadequately investigated in rheumatic disorders, especially in pediatric patients.

METHODS

In this cross-sectional, prospective, and observational study, children with rheumatic disorders and healthy controls were recruited to analyze the quantity and characteristics of circulating DNT cells using flow cytometry.

RESULTS

Overall, the two study groups did not differ in their total DNT cell pool in the bloodstream. However, the number of αβDNT cells was significantly higher in rheumatic children than that in the controls, whereas the γδDNT cells remained similar. This expansion in the circulating pool of αβDNT cells was comparable across different rheumatic diseases, all showing significant differences from the controls in this regard. Moreover, no significant correlation was found between αβDNT cell numbers and disease activity.

CONCLUSIONS

These preliminary results indicate that circulating αβDNT cells are significantly expanded in children with rheumatic disorders; however, this finding appears to be a nonspecific (disease-unrelated) marker of autoimmunity. Further and larger studies are necessary to better investigate and define the role of DNT cells in pediatric rheumatic diseases.

摘要

背景

表达αβT细胞受体的双阴性T(DNT)细胞数量增加是自身免疫性淋巴增殖综合征(ALPS)的诊断标准之一。此外,在一种广泛使用的狼疮小鼠模型中,这些细胞会扩增。然而,在风湿性疾病中,尤其是儿科患者中,αβDNT细胞的体内平衡仍未得到充分研究。

方法

在这项横断面、前瞻性观察研究中,招募了患有风湿性疾病的儿童和健康对照,使用流式细胞术分析循环DNT细胞的数量和特征。

结果

总体而言,两个研究组血液中的总DNT细胞库没有差异。然而,风湿性疾病患儿的αβDNT细胞数量显著高于对照组,而γδDNT细胞数量相似。αβDNT细胞循环池的这种扩增在不同的风湿性疾病中相当,在这方面均与对照组存在显著差异。此外,未发现αβDNT细胞数量与疾病活动之间存在显著相关性。

结论

这些初步结果表明,患有风湿性疾病的儿童循环中的αβDNT细胞显著扩增;然而,这一发现似乎是自身免疫的非特异性(与疾病无关)标志物。需要进一步开展更大规模的研究,以更好地研究和界定DNT细胞在儿童风湿性疾病中的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/11634929/d54cd3ae3b45/12519_2024_854_Fig1_HTML.jpg

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