Kartau Marge, Verkkoniemi-Ahola Auli, Paetau Anders, Palomäki Maarit, Janes Rita, Ristola Matti, Lappalainen Maija, Anttila Veli-Jukka
Clinical Neurosciences, Neurology, Helsinki University Hospital and Helsinki University, Finland.
Department of Pathology (Neuropathology), Helsinki University Hospital Laboratory (HUSLAB), Helsinki University Hospital and Helsinki University, Finland.
Open Forum Infect Dis. 2019 Feb 22;6(2):ofz024. doi: 10.1093/ofid/ofz024. eCollection 2019 Feb.
The aim of this study was to assess the prevalence, incidence rate (IR), predisposing factors, survival rate, and diagnostic delay of progressive multifocal leukoencephalopathy (PML) across medical specialties. Another objective was to survey how PML diagnosis was made in the studied cases.
This is a cross-sectional retrospective observational study of PML cases across different medical specialties during 2004-2016 in the Finnish Capital Region and Southern Finland. Data were obtained from clinical records, clinical microbiology, pathology and radiology department records, and human immunodeficiency virus (HIV) quality register medical records.
A total of 31 patients were diagnosed with PML. The prevalence of PML was 1.56 per 100 000 people and the IR was 0.12 per 100 000 individuals per year during 2004-2016. Hematologic malignancies (n = 19) and HIV/acquired immune deficiency syndrome (n = 5) were the most common underlying diseases, and all patients who had malignant diseases had received cancer treatment. Before PML diagnosis, 21 (67.7%) patients were treated with chemotherapy, 14 (45.2%) patients with rituximab, and 1 patient (3.2%) with natalizumab. Two patients (6.5%) had no obvious immunocompromising disease or treatment. Neither gender, age, first symptoms, previous medication, nor underlying disease influenced the survival of PML patients significantly. The 5-year survival rate was poor, at less than 10%.
The majority of PML patients in our study had a predisposing disease or had immunosuppressive or monoclonal antibody therapy. In the future, broader use of immunosuppressive and immunomodulatory medications may increase incidence of PML among patients with diseases unassociated with PML. Safety screening protocols for John Cunningham virus and PML are important to prevent new PML cases.
本研究旨在评估多灶性白质脑病（PML）在各医学专科中的患病率、发病率、诱发因素、生存率及诊断延迟情况。另一目的是调查所研究病例中PML的诊断方式。
这是一项对2004年至2016年芬兰首都地区和芬兰南部不同医学专科的PML病例进行的横断面回顾性观察研究。数据来自临床记录、临床微生物学、病理学和放射学部门记录以及人类免疫缺陷病毒（HIV）质量登记医疗记录。
共诊断出31例PML患者。2004年至2016年期间，PML的患病率为每10万人中1.56例，发病率为每年每10万人中0.12例。血液系统恶性肿瘤（n = 19）和HIV/获得性免疫缺陷综合征（n = 5）是最常见的基础疾病，所有患有恶性疾病的患者均接受过癌症治疗。在PML诊断之前，21例（67.7%）患者接受过化疗，14例（45.2%）患者接受过利妥昔单抗治疗，1例（3.2%）患者接受过那他珠单抗治疗。2例（6.5%）患者无明显的免疫功能低下疾病或治疗史。性别、年龄、首发症状、既往用药及基础疾病均未对PML患者的生存产生显著影响。5年生存率较差，低于10%。
我们研究中的大多数PML患者有诱发疾病或接受过免疫抑制或单克隆抗体治疗。未来，免疫抑制和免疫调节药物的更广泛使用可能会增加与PML无关疾病患者中PML的发病率。对JC病毒和PML进行安全筛查方案对于预防新的PML病例很重要。
