普通可变免疫缺陷（CVID）合并自身免疫表现患者的淋巴细胞改变
Lymphocyte alterations in patients with Common Variable Immunodeficiency (CVID) and autoimmune manifestations.
作者信息
Rossi Stefano, Baronio Manuela, Gazzurelli Luisa, Tessarin Giulio, Baresi Giulia, Chiarini Marco, Moratto Daniele, Badolato Raffaele, Plebani Alessandro, Lougaris Vassilios
机构信息
Paediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, ASST- Spedali Civili of Brescia, Brescia, Italy.
Flow Cytometry Unit, Clinical Chemistry Laboratory, ASST Spedali Civili di Brescia, Brescia, Italy.
出版信息
Clin Immunol. 2022 Aug;241:109077. doi: 10.1016/j.clim.2022.109077. Epub 2022 Jul 14.
INTRODUCTION
Autoimmunity is a common feature in CVID patients. To date the mechanisms leading to the development of such complications are not fully elucidated.
MATERIALS AND METHODS
Data from 122 CVID patients subdivided in three groups based on the absence of autoimmunity (n-AI) or the presence of hematologic autoimmune phenomena (Cy-AI) or non-hematologic autoimmune phenomena (n-Cy-AI) were evaluated.
RESULTS
We identified a total of 128 autoimmune manifestations in 55/122 patients (45.1%). 30/122 (24.6%) patients presented hematologic autoimmune phenomena while 29/122 (23.8%) presented gastrointestinal autoimmune involvement. Immune thrombocytopenia was the most common manifestation (27/122; 22.1%), followed by autoimmune hemolytic anemia (18/122; 14.8%) and autoimmune enteropathy (17/122; 13.9%). Cy-AI patients displayed higher CD4 effector memory and terminally differentiated CD8 cells with lower percentages of naïve and recent thymic emigrants (RTEs) CD4 cells and a significant expansion of the CD19CD21 population.
CONCLUSIONS
CVID patients developing autoimmune cytopenias display characteristic immune phenotypic features.
引言
自身免疫是常见可变免疫缺陷（CVID）患者的一个常见特征。迄今为止，导致此类并发症发生的机制尚未完全阐明。
材料与方法
评估了122例CVID患者的数据，这些患者根据是否存在自身免疫（无自身免疫，n-AI）或存在血液学自身免疫现象（Cy-AI）或非血液学自身免疫现象（n-Cy-AI）分为三组。
结果
我们在55/122例患者（45.1%）中总共发现了128种自身免疫表现。30/122例（24.6%）患者出现血液学自身免疫现象，而29/122例（23.8%）患者出现胃肠道自身免疫累及。免疫性血小板减少症是最常见的表现（27/122；22.1%），其次是自身免疫性溶血性贫血（18/122；14.8%）和自身免疫性肠病（17/122；13.9%）。Cy-AI患者表现出较高的CD4效应记忆细胞和终末分化的CD8细胞，幼稚和近期胸腺迁出细胞（RTEs）CD4细胞的百分比降低，以及CD19CD21群体的显著扩增。
结论
发生自身免疫性血细胞减少的CVID患者表现出特征性的免疫表型特征。
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