CVID患者B细胞和T细胞表型的评估及其与临床表型的相关性:研究方案

F. T. Zavareh, Y. Bagheri, A. Keshtkar
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引用次数: 3

摘要

背景:常见变异性免疫缺陷(Common variable immunodeficiency, CVID)是最常见的症状性原发性免疫缺陷,其临床表现广泛,从呼吸系统复发性感染到自身免疫、肠病和淋巴细胞增生性疾病。T和B淋巴细胞亚群的一些异常可能与这些临床并发症的发生有关。研究目的:本病例对照研究的主要目的是探讨CVID患者不同淋巴细胞亚群的频率和绝对计数以及细胞增殖反应。确定了淋巴细胞异常与疾病不同临床表型(如单纯感染(IO)、自身免疫(AI)、慢性肠病(CE)和淋巴细胞增生性疾病(LP))之间的相关性。我们还旨在根据淋巴细胞表型来评估CVID的预后。方法:将全外显子组测序(WES)后遗传未解的CVID患者群体细分为IO、AI、CE和LP 4种临床表型,并通过流式细胞术检测等量年龄和性别匹配的健康对照(HC)中CD19+ B细胞、CD4+ T细胞和CD8+ T细胞不同亚群的频率。然后用抗cd3和抗cd28抗体刺激分离的外周血单个核细胞,用羧荧光素琥珀酰基酯(CFSE)试验评估其CD4+ T细胞的增殖反应。使用IBM SPSS Statistics、V.24和Stata软件V.14,根据数据分布的正态性,通过参数检验或非参数检验来评估数据分析。伦理与传播:本研究已获得德黑兰医科大学伦理委员会(ID号:IR.TUMS.VCR.REC.1396.3380)的伦理批准,所有参与者将被要求签署知情同意声明。由于变量、目标和问题范围广泛,本研究的结果将在同行评议的期刊上发表,并在国内和国际会议上发表。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Evaluation of B cell and T cell Phenotypes in CVID Patients and its Correlation with the Clinical Phenotypes: Study Protocol
Background: Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency, which manifests a wide range of clinical phenotypes from recurrent infections of the respiratory system to autoimmunity, enteropathy and lymphoproliferative disorders. Some abnormalities in T and B lymphocyte subpopulations may associate with the development of such clinical complications. Aim of study: The main objective of this case-control study is to investigate the frequency and absolute count of different lymphocyte subsets in CVID patients as well as the cellular proliferation response. Correlation between lymphocyte abnormalities and different clinical phenotypes of the disease such as infection only (IO), autoimmunity (AI), chronic enteropathy (CE) and lymphoproliferative disorders (LP) are determined. We also aim to evaluate the prognosis of CVID for each clinical manifestation based on lymphocyte phenotype. Methods: A population of genetically unsolved CVID patients after whole exome sequencing (WES) will be subdivided into 4 clinical phenotypes i.e. IO, AI, CE and LP and an equal number of age and sex-matched healthy controls (HC) will be examined for the frequency of distinct subgroups of CD19+ B cells, CD4+ T cells and CD8+ T cells by flow cytometry. The proliferation response of their CD4+ T cells is then evaluated by Carboxyfluorescein succinimidyl ester (CFSE) test, using stimulation of isolated peripheral blood mononuclear cells with anti-CD3 and anti-CD28 antibodies. Data analysis will be assessed by parametric or nonparametric tests based on normality of data distribution using IBM SPSS Statistics, V.24 and Stata software V.14. Ethics and dissemination: Ethical approval of this study is received from the Ethics Committee of Tehran University of Medical Sciences (ID number: IR.TUMS.VCR.REC.1396.3380) and all participants will be asked to sign the informed consent statement. Due to the wide range of variables, objectives and questions, the findings of this study are intended to release as multiple publications in peer-reviewed journals and presented at national and international conferences.
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