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Progressive multifocal leukoencephalopathy.

机译:进步多焦点白血病。

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摘要

Progressive multifocal leukoencephalopathy (PML) is a disease of the central nervous system (CNS) with destructive infection of oligodendrocytes by JC virus. PML belongs to the opportunistic infections. It is observed in patients with HIV infection, lymphoid malignancies, after organ- and stem cell transplantations and more recently in the context of modern immune-therapies with monoclonal antibodies (mAb) like natalizumab, rituximab, infliximab and efalizumab. The natural course of PML is fatal within months. More recently, the Immune Reconstitution Inflammatory Syndrome (IRIS) has been observed in patients with HIV infection treated with combination antiretroviral therapy (cART) as well as patients in whom the PML-inducing immune therapy has been terminated. In PML-IRIS the immune system contributes to the elimination of JC virus from the CNS and if PML-IRIS emerges, PML can be survived but can lead as well to catastrophic outcomes with brain herniation and death. Therefore the management of IRIS requires special knowledge in JC virus biology and patient care. JC virus infection is possibly involved in a variety of additional neurological conditions and cancer. Much will be learned within the next years that could change our view on the understanding of JC virus and human disease.
机译:渐进式多焦点白血病(PML)是通过JC病毒具有破坏性感染少突茂细胞的中枢神经系统(CNS)的疾病。 PML属于机会主义的感染。在艾滋病毒感染,淋巴病恶性肿瘤的患者中观察到,在与单克隆抗体(MAB)类似NaTalizumab,Rituximab,Infiximab和Efalizumab的现代免疫疗法的背景下更近于现代免疫疗法的背景下。 PML的自然过程在几个月内致命。最近,在用组合抗逆转录病毒治疗(推车)治疗的HIV感染患者中,观察到免疫重建炎症综合征(IRIS),以及PML诱导免疫治疗终止的患者。在PML - 虹膜中,免疫系统从CNS消除了JC病毒,如果PML-IRIS出现,PML可以存活,但也可以带来脑疝和死亡的灾难性结果。因此,IRI的管理需要JC病毒生物学和患者护理的特殊知识。 JC病毒感染可能涉及各种额外的神经系统病症和癌症。在下一年内将学到很多可能改变我们对对JC病毒和人类疾病的理解的看法。

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