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病例报告:一名同时患有克罗恩病和孤立性先天性脾缺如患者外周血单个核细胞的单细胞图谱。

Case report: Single-cell mapping of peripheral blood mononuclear cells from a patient with both Crohn's disease and isolated congenital asplenia.

机构信息

Department of Gastroenterology, The Second Affiliated Hospital of Zhengzhou University, Zhengzhou, China.

Department of Microbiology and Immunology, School of Basic Medical Sciences, Zhengzhou University, Zhengzhou, China.

出版信息

Front Immunol. 2022 Aug 26;13:959281. doi: 10.3389/fimmu.2022.959281. eCollection 2022.

Abstract

Crohn's disease (CD), as one of the principal form of inflammatory bowel disease (IBD), is characterized by the chronic and recurring inflammatory conditions in the intestine resulting from the over-activation of intestinal immunity. Hyposplenism is strongly associated with CD, while the effect of human spleen on the differentiation and development of immune cell subsets in CD patients remains unclear. Isolated congenital asplenia (ICA) is an extremely rare condition characterized by the absence of a spleen at birth without any other developmental defects. Here, we describe the first case of a patient with both ICA and CD, and follow the progression of CD from remission to active stage. Using cytometry by time of flight (CyTOF) analysis, we draw the first single-cell mapping of peripheral blood mononuclear cells (PBMC) from this unique patient, tracing back to the innate or adaptive immune cell subsets and cell surface markers affected by the spleen. Based on our analysis, it is speculated that the spleen contributes to maintaining immune homeostasis, alleviating intestinal inflammation and improving prognosis by influencing the differentiation and development of peripheral immune cell subsets and the expression of cell surface markers in patients with CD.

摘要

克罗恩病(CD)是炎症性肠病(IBD)的主要形式之一,其特征是肠道免疫过度激活导致的慢性和复发性炎症。脾功能低下与 CD 密切相关,而人类脾脏对 CD 患者免疫细胞亚群的分化和发育的影响尚不清楚。孤立性先天性无脾症(ICA)是一种极其罕见的疾病,其特征是出生时脾脏缺失,无任何其他发育缺陷。在这里,我们描述了首例 ICA 合并 CD 的患者,并观察了 CD 从缓解期到活动期的进展。通过时间飞行(CyTOF)分析,我们对来自该独特患者的外周血单个核细胞(PBMC)进行了首次单细胞图谱绘制,追溯到受脾脏影响的固有或适应性免疫细胞亚群和细胞表面标记物。基于我们的分析,推测脾脏通过影响外周免疫细胞亚群的分化和发育以及 CD 患者细胞表面标记物的表达,有助于维持免疫稳态、减轻肠道炎症和改善预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9224/9459022/dbe29e78e5fa/fimmu-13-959281-g001.jpg

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