Neil Elizabeth C, DeAngelis Lisa M
Department of Neurology, Memorial Sloan Kettering Cancer Center, New York, NY.
Blood Adv. 2017 Oct 18;1(23):2041-2045. doi: 10.1182/bloodadvances.2017008201. eCollection 2017 Oct 24.
Progressive multifocal leukoencephalopathy (PML) is an uncommon opportunistic infection with high morbidity and mortality. This is an institutional review board-approved retrospective review of medical records identified by diagnostic coding for PML or John Cunningham virus (JCV) from 2000 to 2015. Inclusion criteria were cerebrospinal fluid (CSF) positive for JCV by polymerase chain reaction or brain biopsy-proven PML in non-HIV patients. There were 16 patients, 12 of whom were men (75%); the median age was 56 years (range, 31-71 years). All had hematologic malignancies (5 [31%] had chronic lymphocytic leukemia, 3 [19%] had acute myeloid leukemia, 3 had [19%] mantle cell lymphoma, and 1 patient each had acute lymphoblastic leukemia, Hodgkin lymphoma, myeloma, or B-cell lymphoma). One patient received no cancer-directed therapy. Of the remaining 15 patients, all received conventional chemotherapy, and 9 (60%) underwent transplant. Thirteen patients (87%) received immunomodulating therapy (predominantly rituximab). The median time from cancer diagnosis to PML diagnosis was 48.5 months. PML was diagnosed a median of 2.1 months from symptom onset; however, the median time to PML diagnosis was 5.4 months for the 4 patients presenting with a cerebellar syndrome. PML was diagnosed by CSF in 12 patients and brain biopsy in 4 following negative CSF test results. Median survival from PML diagnosis was 4.3 months for the 11 patients on treatment and 0.87 months for the 5 without treatment. PML still occurs in patients with hematologic malignancies in the absence of treatment. Twenty-five percent of our patients required brain biopsy for diagnosis, and diagnosis was delayed when the clinical presentation was unusual, such as a cerebellar syndrome.
进行性多灶性白质脑病（PML）是一种罕见的机会性感染，发病率和死亡率都很高。这是一项经机构审查委员会批准的回顾性研究，对2000年至2015年通过PML或约翰·坎宁安病毒（JCV）诊断编码确定的病历进行回顾。纳入标准为非HIV患者脑脊液（CSF）经聚合酶链反应检测JCV呈阳性或经脑活检证实为PML。共有16例患者，其中12例为男性（75%）；中位年龄为56岁（范围31 - 71岁）。所有患者均患有血液系统恶性肿瘤（5例[31%]患有慢性淋巴细胞白血病，3例[19%]患有急性髓系白血病，3例[19%]患有套细胞淋巴瘤，1例分别患有急性淋巴细胞白血病、霍奇金淋巴瘤、骨髓瘤或B细胞淋巴瘤）。1例患者未接受针对癌症的治疗。其余15例患者均接受了传统化疗，9例（60%）接受了移植。13例患者（87%）接受了免疫调节治疗（主要是利妥昔单抗）。从癌症诊断到PML诊断的中位时间为48.5个月。PML从症状出现到诊断的中位时间为2.1个月；然而，4例出现小脑综合征的患者诊断为PML的中位时间为5.4个月。12例患者通过CSF诊断为PML，4例CSF检测结果为阴性后经脑活检确诊。11例接受治疗的患者从PML诊断后的中位生存期为4.3个月，5例未接受治疗的患者为0.87个月。在未接受治疗的血液系统恶性肿瘤患者中仍会发生PML。我们25%的患者需要进行脑活检以明确诊断，当临床表现不典型（如小脑综合征）时诊断会延迟。
