迟发性联合免疫缺陷伴进行性多灶性白质脑病及相关免疫重建炎症综合征
Late-Onset Combined Immunodeficiency presenting with Progressive Multifocal Leukoencephalopathy and associated Immune Reconstitution Inflammatory Syndrome.
作者信息
Mezzadri Luca, Borghesi Maria Lucia, Comoli Patrizia, Pozzi Maria Rosa, Lapadula Giuseppe, Rossi Marianna, Bonfanti Paolo
机构信息
Infectious Diseases Unit, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.
School of Medicine and Surgery, University of Milano-Bicocca, Milan, Italy.
出版信息
Infez Med. 2025 Sep 1;33(3):333-338. doi: 10.53854/liim-3303-11. eCollection 2025.
Abstract
Progressive multifocal leukoencephalopathy (PML) can rarely occur in individuals with occult immunosuppression. Here, we describe the case of an adult man who presented with PML, in whom CD4+ lymphocytopenia and hypogammaglobulinemia were subsequently identified, leading to a diagnosis of late-onset combined immunodeficiency. Intravenous immunoglobulin replacement therapy was initiated. His clinical course, though complicated by immune reconstitution inflammatory syndrome, was favorable.
摘要
进行性多灶性白质脑病（PML）很少发生于隐匿性免疫抑制个体。在此，我们描述一例成年男性患者，该患者表现为PML，随后发现其存在CD4 +淋巴细胞减少和低丙种球蛋白血症，最终诊断为迟发性联合免疫缺陷。遂开始静脉注射免疫球蛋白替代治疗。尽管该患者的临床病程因免疫重建炎症综合征而复杂化，但总体预后良好。
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