Division of Human Immunology, SA Pathology, University of Adelaide, Adelaide, Australia.
Clin Exp Immunol. 2012 Feb;167(2):275-81. doi: 10.1111/j.1365-2249.2011.04507.x.
Abnormalities in peripheral blood B cell subsets have been identified in common variable immunodeficiency (CVID) patients and classification systems based upon their numbers have been proposed to predict the clinical features. We analysed B lymphocyte subsets by multi-colour flow cytometry (MFC) in a cohort of well-characterized CVID patients to look at their clinical relevance and validate the published association of different classification criteria (Freiburg, Paris and Euroclass) with clinical manifestations. CVID patients had a reduced proportion of total and switched memory B cells (MBC, swMBC) compared to normal controls (P < 0·0006). Patients classified in Freiburg Ia had a higher prevalence of granulomatous diseases (P = 0·0034). The previously published associations with autoimmune diseases could not be confirmed. The Euroclass classification was not predictive of clinical phenotypes. The absolute numbers of all B cell subsets were reduced in CVID patients compared to controls. There was a significant linear correlation between low absolute total B cells and MBC with granulomatous disease (P < 0·05) and a trend towards lower B cells in patients with autoimmune diseases (P = 0·07). Absolute number of different B cell subsets may be more meaningful than their relative percentages in assessing the risk of granulomatous diseases and possibly autoimmunity.
外周血 B 细胞亚群异常已在普通变异性免疫缺陷(CVID)患者中得到鉴定,并且已经提出了基于其数量的分类系统来预测临床特征。我们通过多色流式细胞术(MFC)对一组特征明确的 CVID 患者的 B 淋巴细胞亚群进行了分析,以研究其临床相关性,并验证不同分类标准(弗莱堡、巴黎和 Euroclass)与临床表现之间的已发表关联。与正常对照相比,CVID 患者的总和转换记忆 B 细胞(MBC、swMBC)比例降低(P < 0·0006)。在 Freiburg Ia 分类的患者中,肉芽肿性疾病的患病率更高(P = 0·0034)。先前与自身免疫性疾病相关的关联无法得到证实。Euroclass 分类不能预测临床表型。与对照组相比,CVID 患者所有 B 细胞亚群的绝对数量均减少。绝对总 B 细胞和 MBC 与肉芽肿性疾病呈显著线性相关(P < 0·05),而自身免疫性疾病患者的 B 细胞数量呈下降趋势(P = 0·07)。在评估肉芽肿性疾病和可能的自身免疫风险时,B 细胞不同亚群的绝对数量可能比其相对百分比更有意义。