No evidence of beneficial effects of plasmapheresis in natalizumab-associated PML

No evidence of beneficial effects of plasmapheresis in natalizumab-associated PML
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DOI:
10.1212/wnl.0000000000003740
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发表时间:
2017-03-21
期刊:
影响因子:
9.9
通讯作者:
Centonze, Diego
Centonze, Diego
中科院分区:
医学1区
文献类型:
--
作者:
Landi, Doriana;De Rossi, Nicola;Centonze, Diego

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目的:回顾分析血浆置换(PLEX)对多发性硬化症(MS)和奈他珠单抗(NTZ)相关性进行性多灶性白质脑病(PML)患者生存率和临床结局的影响。总共包括193个国际案例和34个意大利案例。临床结果是通过比较患者在PML诊断时的临床状态和PML缓解后的状态来确定的。单因素和多因素分析研究PLEX、性别、年龄、国家、PML前扩展残疾状态量表评分、NTZ输注次数、既往免疫抑制剂暴露、PML症状、PML病变部位、脑脊液JC病毒状态和复制、附加PML治疗和类固醇治疗以及PML免疫重建炎症综合征(IRIS)的进展对生存率和临床预后的影响。结果:共分析219例NPZ-PML病例,其中184例(84%)接受了PLEX,这并不能降低死亡风险或预后差与好的可能性。结论:Plex不能改善意大利或国际MS和NTZ-PML患者的存活率或临床结果,提示今后应谨慎进行该治疗。证据分类:这项研究提供了III类证据,证明对于患有NTZ-PML的患者,PLEX不能提高存活率。这项研究缺乏统计精确度,无法排除PLEX的重要益处或危害。
Objective: To examine retrospectively the effects of plasmapheresis (PLEX) on the survival and clinical outcomes of patients with multiple sclerosis (MS) and natalizumab (NTZ)-associated progressive multifocal leukoencephalopathy (PML).Methods: The medical literature was searched for the terms natalizumab and progressive multifocal leukoencephalopathy. A total of 193 international and 34 Italian NTZ-PML cases were included. Clinical outcome was determined by comparing the patients' clinical status at PML diagnosis with status after PML resolution. The effects on survival and clinical outcome of PLEX, sex, age, country, pre-PML Expanded Disability Status Scale score, NTZ infusion number, prior immunosuppressant exposure, PML symptoms, PML lesion location at diagnosis, CSF JC virus status and copies, additional PML treatments and steroids, and PML immune reconstitution inflammatory syndrome ( IRIS) development were investigated with both univariate and multivariate analyses.Results: A total of 219 NTZ-PML cases were analyzed, and 184 (84%) underwent PLEX, which did not reduce the mortality risk or the likelihood of poor vs favorable outcomes. Country was predictive of mortality and poor outcome, while PML-IRIS development was predictive of poor outcome.Conclusions: PLEX did not improve the survival or clinical outcomes of Italian or international patients with MS and NTZ-PML, suggesting that this treatment should be performed cautiously in the future. Classification of evidence: This study provides Class III evidence that for patients with NTZ-PML, PLEX does not improve survival. The study lacks the statistical precision to exclude an important benefit or harm of PLEX.