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[儿童原发性免疫性血小板减少症与结缔组织病早期鉴别的临床和免疫学特征]

[Clinical and immunological features for early differentiation between primary immune thrombocytopenia and connective tissue disease in children].

作者信息

Kang Fu-Rong, Yan Mei, Yue Ying-Bin, Nuriddin Hailiguli, Cheng Yong-Feng, Liu Yu

机构信息

Pediatric Center, The First Affiliated Hospital of Xinjiang Medical University, Urumqi 830054, China.

出版信息

Zhongguo Dang Dai Er Ke Za Zhi. 2025 Aug 15;27(8):974-981. doi: 10.7499/j.issn.1008-8830.2411121.

DOI:10.7499/j.issn.1008-8830.2411121
PMID:40831167
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12369532/
Abstract

OBJECTIVES

To investigate the clinical and immunological features of children with primary immune thrombocytopenia (pITP) or connective tissue disease (CTD) with thrombocytopenia as the initial manifestation at initial diagnosis, and to provide a basis for early differentiation.

METHODS

A retrospective study was performed on 236 children with pITP (pITP group) or CTD with thrombocytopenia as the initial manifestation (CTD-TP group) who were admitted from January 2019 to August 2024. Clinical and immunological indicators were compared between the two groups to identify potential influencing factors for early differentiation and their discriminative validity.

RESULTS

Compared with the pITP group, the CTD-TP group had a significantly older age of onset and significantly lower leukocyte count, eosinophil count, lymphocyte count, and complement C4 level (<0.05), as well as significantly higher levels of C-reactive protein, IgE, and IgM (<0.05). The logistic regression analysis showed that age, IgE, IgM, total B cells, and complement C4 were predictive factors for early differentiation between pITP and CTD-TP (<0.05). The receiver operating characteristic curve analysis showed that a combination of these five factors had a good discriminative validity, with an area under the curve of 0.944. The correlation analysis showed a negative correlation between IgG and platelet count in the pITP group (=-0.363, <0.05) and a positive correlation between NK cells and platelet count in the CTD-TP group (=0.713, <0.05).

CONCLUSIONS

There is heterogeneity in the clinical and immunological indicators between children with pITP and CTD-TP at initial diagnosis, and these research findings can help with the early differentiation between the two diseases.

摘要

目的

探讨以血小板减少为初发表现的原发性免疫性血小板减少症(pITP)或结缔组织病(CTD)患儿在初诊时的临床及免疫学特征,为早期鉴别提供依据。

方法

对2019年1月至2024年8月收治的236例以血小板减少为初发表现的pITP患儿(pITP组)或CTD患儿(CTD-TP组)进行回顾性研究。比较两组的临床及免疫学指标,以确定早期鉴别的潜在影响因素及其判别效度。

结果

与pITP组相比,CTD-TP组发病年龄显著偏大,白细胞计数、嗜酸性粒细胞计数、淋巴细胞计数及补体C4水平显著降低(<0.05),而C反应蛋白、IgE及IgM水平显著升高(<0.05)。Logistic回归分析显示,年龄、IgE、IgM、总B细胞及补体C4是pITP与CTD-TP早期鉴别的预测因素(<0.05)。受试者工作特征曲线分析显示,这五个因素联合具有良好的判别效度,曲线下面积为0.944。相关性分析显示,pITP组IgG与血小板计数呈负相关(r=-0.363,<0.05),CTD-TP组NK细胞与血小板计数呈正相关(r=0.713,<0.0

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