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评估成人普通变异型免疫缺陷患者合并免疫缺陷的实验室标准。

Evaluating laboratory criteria for combined immunodeficiency in adult patients diagnosed with common variable immunodeficiency.

机构信息

Department for Rheumatology and Clinical Immunology and Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany; Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany.

Department for Rheumatology and Clinical Immunology and Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany.

出版信息

Clin Immunol. 2019 Jun;203:59-62. doi: 10.1016/j.clim.2019.04.001. Epub 2019 Apr 17.

Abstract

Some patients diagnosed with common variable immunodeficiency (CVID) actually suffer from combined immunodeficiency (CID) and therefore may require a different, CID-adapted treatment. Several CD4 T-cell-based criteria have been proposed in the past to identify patients with CID within the cohort of adult CVID patients. In this monocentric study, we used retrospective immunological and clinical data of 238 CVID patients to compare four different proposals of how to define CID among CVID patients. We demonstrate that none of the current definitions sufficiently separates CID from CVID patients and that the relative reduction of naïve CD4 T cells <10% has the highest sensitivity of all tested markers for patients with clinical complications often associated with CID. Thus, a very low percentage of naïve CD4 T cells in any adult CVID patient should raise suspicion, but is not sufficient to define CID among CVID patients.

摘要

一些被诊断为普通变异性免疫缺陷症(CVID)的患者实际上患有联合免疫缺陷症(CID),因此可能需要不同的、适应 CID 的治疗方法。过去曾提出过几种基于 CD4 T 细胞的标准,以在成年 CVID 患者队列中识别出患有 CID 的患者。在这项单中心研究中,我们使用了 238 例 CVID 患者的回顾性免疫学和临床数据,比较了四种不同的方法来定义 CID 在 CVID 患者中的情况。我们证明,目前的任何一种定义都不能充分地区分 CID 和 CVID 患者,并且幼稚 CD4 T 细胞相对减少<10%是所有测试标志物中对与 CID 相关的临床并发症患者的敏感性最高的标志物。因此,任何成年 CVID 患者中幼稚 CD4 T 细胞的极低百分比都应引起怀疑,但不足以在 CVID 患者中定义 CID。

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