First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Department of Neurology, Laikon General Hospital, Athens, Greece.
Eur J Haematol. 2022 May;108(5):359-368. doi: 10.1111/ejh.13751. Epub 2022 Feb 6.
Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal demyelinating disease of the central nervous system (CNS) caused by the reactivation of JC polyomavirus in the CNS. We present a case of a 54-year-old man with follicular lymphoma diagnosed with PML after being treated with anti-CD20 monoclonal antibody-based regimens for several years. Due to the lack of effective treatment choices for PML, the patient was treated with nivolumab, based on recent reports, but succumbed to his disease a few months after diagnosis. In this paper, we focus on reviewing the literature of PML cases correlated with newer agents used in hematology, possible factors affecting disease prognosis, as well as the available data on upcoming therapeutic options for patients with PML. Though newer promising treatments such as anti-PD1 monoclonal antibodies arise, a definitive treatment option is yet to be found. Vigilance, early detection, and prompt intervention play a crucial role in the prognosis of PML in patients with hematological malignancies.
进行性多灶性白质脑病(PML)是一种罕见的、常致命的中枢神经系统(CNS)脱髓鞘疾病,由 CNS 中 JC 多瘤病毒的再激活引起。我们报告了一例 54 岁男性滤泡性淋巴瘤患者的病例,该患者在接受抗 CD20 单克隆抗体方案治疗数年后被诊断为 PML。由于缺乏有效的 PML 治疗选择,根据最近的报告,患者接受了nivolumab 治疗,但在诊断后几个月内死于该病。在本文中,我们重点回顾了与血液学中使用的新型药物相关的 PML 病例的文献,可能影响疾病预后的因素,以及针对 PML 患者的现有治疗选择的数据。尽管出现了更新的有希望的治疗方法,如抗 PD1 单克隆抗体,但仍未找到明确的治疗选择。在血液恶性肿瘤患者中,警惕、早期发现和及时干预对 PML 的预后起着至关重要的作用。