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肾移植后进行性多灶性白质脑病免疫抑制剂治疗撤药后的良好结局:病例报告及文献综述

Favorable outcome after withdrawal of immunosuppressant therapy in progressive multifocal leukoencephalopathy after renal transplantation: case report and literature review.

作者信息

Ohara Hiroya, Kataoka Hiroshi, Nakamichi Kazuo, Saijo Masayuki, Ueno Satoshi

机构信息

Department of Neurology, Nara Medical University, Kashihara, Nara, Japan.

Department of Neurology, Nara Medical University, Kashihara, Nara, Japan.

出版信息

J Neurol Sci. 2014 Jun 15;341(1-2):144-6. doi: 10.1016/j.jns.2014.03.048. Epub 2014 Apr 2.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease caused by the JC polyomavirus (JCV). Most patients with PML after renal transplantation have had poor outcomes. We describe a patient with PML after renal transplantation who had a good response to the withdrawal of immunosuppressant therapy. We performed quantitative real-time PCR testing for JCV DNA in cerebrospinal fluid (CSF), and assessed mutation of the JC virus genome detected in the CSF. At the same time, we checked cranial magnetic resonance imaging (MRI). Immunosuppressant therapy was discontinued immediately. The MRI scan that followed showed markedly decreased numbers of high intensity signals, and the results of real-time PCR for JCV DNA in CSF became negative. The patient had no other neurological deficits. Withdrawal of immunosuppressant treatment has a beneficial effect on the course of PML after renal transplantation, and quantitative PCR may facilitate the immediate withdrawal of immunosuppressant agents.

摘要

进行性多灶性白质脑病(PML)是一种由JC多瘤病毒(JCV)引起的致命性脱髓鞘疾病。大多数肾移植后发生PML的患者预后较差。我们描述了一名肾移植后发生PML的患者,其对停用免疫抑制治疗有良好反应。我们对脑脊液(CSF)中的JCV DNA进行了定量实时PCR检测,并评估了在CSF中检测到的JC病毒基因组的突变。同时,我们检查了头颅磁共振成像(MRI)。立即停用免疫抑制治疗。随后的MRI扫描显示高强度信号数量明显减少,CSF中JCV DNA的实时PCR结果变为阴性。该患者没有其他神经功能缺损。停用免疫抑制治疗对肾移植后PML的病程有有益影响,定量PCR可能有助于立即停用免疫抑制剂。

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