进行性多灶性白质脑病:当前的见解

IF 2.1 Q3 CLINICAL NEUROLOGY Degenerative neurological and neuromuscular disease Pub Date : 2019-12-02 eCollection Date: 2019-01-01 DOI:10.2147/DNND.S203405
Marge Kartau, Jussi Ot Sipilä, Eeva Auvinen, Maarit Palomäki, Auli Verkkoniemi-Ahola
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引用次数: 0

摘要

摘要PML病例应根据易感因素进行评估,因为这些亚组因发病率、临床病程和预后而异。PML风险最高的三个群体是血液系统恶性肿瘤患者、HIV感染患者和使用单克隆抗体(mAb)治疗的患者,这些患者大多以前接受过免疫治疗,但也未接受治疗。流行病学数据稀少,部分相互矛盾,但亚组的分布似乎发生了变化。虽然没有特异性的抗JCPyV治疗,但恢复免疫功能是治疗PML最有效的方法。有必要进行研究以确定免疫检查点抑制剂是否对某些PML亚组有益。除了接受那他珠单抗(NTZ)治疗的MS患者外,没有PML诊断或风险分层算法的系统性国家或国际记录。需要这些措施来改进PML风险评估并制定更好的预防策略。
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Progressive Multifocal Leukoencephalopathy: Current Insights.

Cases of PML should be evaluated according to predisposing factors, as these subgroups differ by incidence rate, clinical course, and prognosis. The three most significant groups at risk of PML are patients with hematological malignancies mostly previously treated with immunotherapies but also untreated, patients with HIV infection, and patients using monoclonal antibody (mAb) treatments. Epidemiological data is scarce and partly conflicting, but the distribution of the subgroups appears to have changed. While there is no specific anti-JCPyV treatment, restoration of the immune function is the most effective approach to PML treatment. Research is warranted to determine whether immune checkpoint inhibitors could benefit certain PML subgroups. There are no systematic national or international records of PML diagnoses or a risk stratification algorithm, except for MS patients receiving natalizumab (NTZ). These are needed to improve PML risk assessment and to tailor better prevention strategies.

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