Vlková M, Thon V, Sárfyová M, Bláha L, Svobodník A, Lokaj J, Litzman J
Department of Clinical Immunology and Allergology, St Anne's University Hospital, Masaryk University, Brno, Czech Republic.
Clin Exp Immunol. 2006 Feb;143(2):373-9. doi: 10.1111/j.1365-2249.2006.02999.x.
Common variable immunodeficiency (CVID) is primary hypogammaglobulinaemia with an unknown aetiopathogenesis. Although various abnormalities of T and B cells have been described, their pathogenetic roles are unclear. We determined T and B lymphocyte subsets known to be abnormal in CVID in order to disclose possible relations between numerical abnormalities in those cells. Markers associated with B cell development (CD21, CD27, IgM, IgD) were determined on B lymphocytes (CD19+); T lymphocyte development (CD45RA, CD45RO, CD62L) and activation markers (CD25, CD27, CD28, CD29, CD38, CD57, HLA-DR) were determined on CD4+ and CD8+ T lymphocytes in 42 CVID patients and in 33 healthy controls. Abnormalities in CD4+ T lymphocyte activation markers (increase in CD29, HLA-DR, CD45RO, decrease in CD27, CD62L, CD45RA) were observed particularly in patients with a decreased number of memory (CD27+) and mature (CD21+) B cells (group Ia according to the Freiburg group's classification), while abnormalities observed in CD8+ cells (increase in CD27 and CD28 and decrease in HLA-DR, CD57 and CD38) did not depend upon grouping patients together according to B lymphocyte developmental subpopulations. We observed correlations between immature B cells (IgM+ CD21-) and expression of CD27, CD62L, CD45RA, CD45RO and HLA-DR on CD4+ T cells in CVID patients but not in the control group. The expression of CD27 and CD45RA on CD4+ T lymphocytes, such as the percentage of IgD+ CD27- and IgD+ CD27+ cells in B lymphocytes, showed age dependency to be more significant than in the control group. Our study demonstrates that T and B lymphocyte abnormalities in CVID are partially related to each other. Some of those abnormalities are not definite, but may evolve with age of the patient.
普通变异型免疫缺陷(CVID)是一种病因不明的原发性低丙种球蛋白血症。尽管已描述了T细胞和B细胞的各种异常,但其致病作用尚不清楚。我们测定了已知在CVID中异常的T和B淋巴细胞亚群,以揭示这些细胞数量异常之间可能存在的关系。在B淋巴细胞(CD19 +)上测定与B细胞发育相关的标志物(CD21、CD27、IgM、IgD);在42例CVID患者和33名健康对照者的CD4 +和CD8 + T淋巴细胞上测定T淋巴细胞发育(CD45RA、CD45RO、CD62L)和活化标志物(CD25、CD27、CD28、CD29、CD38、CD57、HLA - DR)。特别是在记忆(CD27 +)和成熟(CD21 +)B细胞数量减少的患者(根据弗莱堡组分类为Ia组)中观察到CD4 + T淋巴细胞活化标志物异常(CD29、HLA - DR、CD45RO增加,CD27、CD62L、CD45RA减少),而在CD8 +细胞中观察到的异常(CD27和CD28增加,HLA - DR、CD57和CD38减少)并不取决于根据B淋巴细胞发育亚群对患者进行分组。我们观察到CVID患者中未成熟B细胞(IgM + CD21 -)与CD4 + T细胞上CD27、CD62L、CD45RA、CD45RO和HLA - DR的表达之间存在相关性,而在对照组中未观察到这种相关性。CD4 + T淋巴细胞上CD27和CD45RA的表达,以及B淋巴细胞中IgD + CD27 -和IgD + CD27 +细胞的百分比,显示出年龄依赖性,且比对照组更显著。我们的研究表明,CVID中的T和B淋巴细胞异常部分相互关联。其中一些异常并不确定,但可能随患者年龄而演变。