Fulcher D A, Avery D T, Fewings N L, Berglund L J, Wong S, Riminton D S, Adelstein S, Tangye S G
Immunology Unit, Institute of Clinical Pathology and Medical Research, Westmead Hospital, Westmead, Sydney, NSW 2145, Australia.
Clin Exp Immunol. 2009 Sep;157(3):365-9. doi: 10.1111/j.1365-2249.2009.03973.x.
Common variable immunodeficiency (CVID) is a B cell immunodeficiency disorder characterized frequently by failure of memory B cell development and antibody secretion. A unifying cellular pathogenesis for CVID has not been forthcoming, but given the immunoregulatory role of invariant NK (iNK) T cells and their absence in several other immunodeficiencies, we quantified these cells in the blood of 58 CVID patients. There was a marked decrease in the proportion of iNK T cells in CVID patients compared with controls. This was particularly notable in those with low isotype-switched memory B cells, but subset analysis demonstrated no difference when stratified by specific clinical features. We propose that the decreased proportion of iNK T cells in CVID might be linked to the failure of memory B cell generation, which may contribute to reduced antibody production in these patients.
常见变异型免疫缺陷(CVID)是一种B细胞免疫缺陷疾病,其特征通常为记忆B细胞发育和抗体分泌失败。尚未找到CVID统一的细胞发病机制,但鉴于不变自然杀伤(iNK)T细胞的免疫调节作用以及它们在其他几种免疫缺陷中的缺失,我们对58例CVID患者血液中的这些细胞进行了定量分析。与对照组相比,CVID患者中iNK T细胞的比例显著降低。这在低同种型转换记忆B细胞的患者中尤为明显,但亚组分析表明,按特定临床特征分层时无差异。我们提出,CVID中iNK T细胞比例的降低可能与记忆B细胞生成失败有关,这可能导致这些患者抗体产生减少。