Division of Allergy and Immunology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Institute for Immunology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
J Clin Invest. 2020 Oct 1;130(10):5105-5108. doi: 10.1172/JCI141717.
Idiopathic CD4+ T cell lymphocytopenia (ICL) is a heterogeneous syndrome presenting with persistent CD4+ T cell lymphopenia of unknown origin, and opportunistic infections in some patients. The underlying pathogenesis and appropriate management remain understudied. In this issue of the JCI, Perez-Diez and Wong et al. assessed the prevalence of autoantibodies from the sera of 51 adult ICL patients (out of a cohort of 72). Some patients showed high levels of IgG and IgM autoantibodies against numerous autoantigens, and some autoantibodies were specific for lymphocytes. The researchers implicate these autoantibodies as a possible pathogenic mechanism responsible for the reduction in circulating CD4+ T cells. This study goes beyond defining a mechanism in a complex, poorly defined disease; it also brings a renewed focus on ICL that will likely result in improved diagnostic evaluation and treatment.
特发性 CD4+T 细胞淋巴细胞减少症（ICL）是一种异质性综合征，表现为不明原因的持续性 CD4+T 细胞淋巴细胞减少症，并伴有一些患者的机会性感染。其潜在发病机制和适当的治疗方法仍有待研究。在本期 JCI 中，Perez-Diez 和 Wong 等人评估了 51 例成人 ICL 患者（来自 72 例患者队列）血清中的自身抗体的患病率。一些患者表现出针对多种自身抗原的 IgG 和 IgM 自身抗体的高水平，并且一些自身抗体是针对淋巴细胞的。研究人员认为这些自身抗体可能是导致循环 CD4+T 细胞减少的潜在致病机制。这项研究不仅定义了一种复杂且定义不明确的疾病的机制，还重新关注了 ICL，这可能会导致改进的诊断评估和治疗。
