Weber T, Weber F, Petry H, Lüke W
Department of Neurology, Marienkrankenhaus, Hamburg, Germany.
J Neurovirol. 2001 Aug;7(4):311-7. doi: 10.1080/13550280152537166.
Progressive multifocal leukoencephalopathy (PML) is a disease usually occurring in immunosuppressed patients. By far the most common underlying immunosuppressive illness is the acquired immune deficiency syndrome, accounting for about 85% of PML cases currently seen in clinical practice. PML may occur in patients with deficits in the humoral and/or cellular immune response such as lymphoproliferative diseases, myeloproliferative diseases, carcinomatous diseases and acquired immunodeficiency due to autoimmune diseases and immunosuppressive therapy. The humoral immune response in PML is indicative of a persistent, reactivated infection with a prominent immunoglobulin (lgG) G synthesis to virus protein 1 (VP1). An lgM synthesis in serum is rarely found. In about 76% of PML cases, an intrathecal humoral immune response to recombinant VP1 can be found as compared to only 3.2% in healthy controls. The detection of intrathecally synthesized lgG antibodies to VP1 can be used as an additional diagnostic test for the diagnosis of PML. The magnitude of the intrathecal humoral immune response appears to rise over time and may be associated with a decrease of viral load in cerebrospinal fluid (CSF) and possibly the central nervous system (CNS). Compared to healthy controls, proliferation of peripheral blood mononuclear cells (PBMC) is reduced in PML patients. Immunological studies suggest a general impairment of the Th1-type T-helper cell function of cell-mediated immunity. Furthermore, the appearance of JCV-specific cytotoxic T-lymphocytes appears to be associated with a favorable clinical outcome.
进行性多灶性白质脑病（PML）是一种通常发生在免疫抑制患者中的疾病。迄今为止，最常见的潜在免疫抑制性疾病是获得性免疫缺陷综合征，约占目前临床实践中所见PML病例的85%。PML可能发生在体液和/或细胞免疫反应存在缺陷的患者中，如淋巴增殖性疾病、骨髓增殖性疾病、癌性疾病以及自身免疫性疾病和免疫抑制治疗导致的获得性免疫缺陷。PML中的体液免疫反应表明存在持续的、重新激活的感染，对病毒蛋白1（VP1）有显著的免疫球蛋白（IgG）合成。血清中很少发现IgM合成。在约76%的PML病例中，可发现对重组VP1的鞘内体液免疫反应，而健康对照中仅为3.2%。检测鞘内合成的针对VP1的IgG抗体可作为诊断PML的一项额外诊断测试。鞘内体液免疫反应的强度似乎随时间增加，可能与脑脊液（CSF）以及可能的中枢神经系统（CNS）中病毒载量的降低有关。与健康对照相比，PML患者外周血单个核细胞（PBMC）的增殖减少。免疫学研究表明细胞介导免疫的Th1型辅助性T细胞功能普遍受损。此外，JC病毒特异性细胞毒性T淋巴细胞的出现似乎与良好的临床结果相关。
