Sasahara Yoji, Wada Taizo, Morio Tomohiro
Department of Pediatrics, Tohoku University Graduate School of Medicine, Miyagi, Japan.
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Ishikawa, Japan.
Clin Immunol. 2022 Sep;242:109098. doi: 10.1016/j.clim.2022.109098. Epub 2022 Aug 13.
T cells following immunological synapse (IS) formation with antigen-presenting cells produce multiple cytokines through T cell receptor, integrin, and costimulatory signaling. Here, we investigated the cytokine profiles following IS formation in response to staphylococcal superantigen exposure in three adolescent patients with classical Wiskott-Aldrich syndrome (WAS) and in one patient with leukocyte adhesion deficiency (LAD) type 1. All WAS patients showed lower Th1 and Th2-skewed cytokine production; similar results were observed in the flow cytometric analysis of IFNγ- and IL-4-producing T cells. The patient with LAD type 1 with somatic mosaicism in 2% of CD8+ T cells showed lower Th1 and Th2 cytokine production than healthy controls. The patients with WAS were susceptible to infections and atopic manifestations, and the patients with LAD type 1 showed cold abscess on their skin, our findings using patient samples provide clinical insights into the mechanisms underlying immunodeficiency related to the symptoms of each disease.
与抗原呈递细胞形成免疫突触（IS）后，T细胞通过T细胞受体、整合素和共刺激信号产生多种细胞因子。在此，我们研究了3例经典威斯科特-奥尔德里奇综合征（WAS）青少年患者和1例1型白细胞黏附缺陷（LAD）患者在暴露于葡萄球菌超抗原后形成IS后的细胞因子谱。所有WAS患者均表现出较低的Th1和Th2偏向性细胞因子产生；在产生IFNγ和IL-4的T细胞的流式细胞术分析中也观察到了类似结果。1型LAD患者2%的CD8+T细胞存在体细胞镶嵌现象，其Th1和Th2细胞因子产生低于健康对照。WAS患者易发生感染和特应性表现，1型LAD患者皮肤出现冷脓肿，我们使用患者样本的研究结果为每种疾病症状相关免疫缺陷的潜在机制提供了临床见解。
