Department of Advanced Medical and Surgical Sciences, Second Division of Neurology - University of Campania "Luigi Vanvitelli", via Pansini 5, 80131 Naples, Italy.
Department of Advanced Biomedical Sciences, University of Naples "Federico II", via Pansini 5, 80131 Naples, Italy.
J Neuroimmunol. 2020 Dec 15;349:577420. doi: 10.1016/j.jneuroim.2020.577420. Epub 2020 Sep 29.
Idiopathic CD4+ lymphocytopenia (ICL) is a rare disorder characterized by low counts of CD4+ cells (<300/mm) in absence of other known causes of immunosuppression. A few cases of progressive multifocal leukoencephalopathy (PML) were reported in association with ICL with variable outcome. We describe the case of a 40 year-old man diagnosed with PML, which showed a monophasic course. Causes of primary and secondary immunodeficiency were ruled out, only a "borderline" ICL was found. This case highlights that a severe immunodepression could not be an absolute prerequisite in developing PML and also points the attention on current definition of ICL.
特发性 CD4+ 淋巴细胞减少症 (ICL) 是一种罕见疾病，其特征是 CD4+ 细胞计数低（<300/mm），而无其他已知免疫抑制原因。有少数报道称，ICL 与进展性多灶性白质脑病 (PML) 相关，其结果各不相同。我们描述了一例诊断为 PML 的 40 岁男性病例，其表现为单相病程。排除了原发性和继发性免疫缺陷的原因，仅发现“临界”ICL。该病例强调，严重免疫抑制并非发生 PML 的绝对必要条件，并提请注意当前 ICL 的定义。
