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肾移植受者并发暴发性进行性多灶性白质脑病-免疫重建炎症综合征:一种罕见的临床结局及文献复习。

A Kidney Transplant Recipient With Fulminant Progressive Multifocal Leukoencephalopathy-Immune Reconstitution Inflammatory Syndrome: A Rare Clinical Outcome and Review of the Literature.

机构信息

>From the Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, Zagreb, Croatia.

出版信息

Exp Clin Transplant. 2020 Apr;18(2):242-246. doi: 10.6002/ect.2018.0227. Epub 2019 Oct 1.

Abstract

Progressive multifocal leukoencephalopathy is a devastating disease affecting the central nervous system that may be seen in immunocompromised patients. We present a case of a kidney transplant recipient who received tacrolimus, mycophenolic acid, and prednisone and who developed motor deficits, altered cognition, and speech abnormalities, which culminated in a coma. The diagnosis was made by detecting John Cunningham polyomavirus DNA with polymerase chain reaction and observing characteristic findings on magnetic resonance imaging. Soon after immunosuppressive therapy was withdrawn, the patient's clinical status deteriorated due to immune reconstitution inflammatory syndrome, and prednisone was administered. Unfortunately, the patient died about 9 months after onset of symptoms. This case serves to illustrate the fulminant progression of progressive multifocal leukoencephalopathy and the possible complications that may arise when treating it.

摘要

进行性多灶性白质脑病是一种影响中枢神经系统的破坏性疾病,可能发生在免疫功能低下的患者中。我们报告了一例肾移植受者的病例,该受者接受了他克莫司、霉酚酸酯和泼尼松治疗,随后出现运动功能障碍、认知改变和言语异常,并最终导致昏迷。该诊断通过聚合酶链反应检测到约翰·坎宁安多瘤病毒 DNA 并观察磁共振成像上的特征性发现而做出。免疫抑制治疗撤回后不久,患者因免疫重建炎症综合征而出现临床状况恶化,并给予泼尼松治疗。不幸的是,该患者在出现症状后约 9 个月死亡。该病例说明了进行性多灶性白质脑病的迅速进展以及在治疗该病时可能出现的并发症。

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