Sokol Juraj, Lisá Lenka, Zeleňáková Jana, Balhárek Tomáš, Plameňová Ivana, Staško Ján, Kubisz Peter
Vnitr Lek. 2017 Winter;63(1):60-64.
The definition "Progressive Multifocal Leukoencephalopathy" (PML) was first used in 1958 to describe a fatal demyelinating central nervous system (CNS) disease in patients with lymphoproliferative disorders. In 1971, the virus responsible for the disease was isolated and named John Cunningham virus (JCV). We present a rare case of a 62-year-old male with chronic lymphocytic leukemia and PML. In our work, we discuss the diagnostic and therapeutic challenges and offer suggestions for preventing PML development. The main learning points are: 1. Regularly check the level of immunoglobulins and the CD4+ : CD8+ T-cell ratio, intravenous administration of immunoglobulins should be considered when recording their reduction. 2. In checking the CD4+ : CD8+ T-cell ratio and verifying the impossibility of raising the level of immunoglobulins, we must weigh the possible benefits of continuing treatment with monoclonal antibody against the risks. 3. Physicians should maintain a high index of suspicion for the development of PML in patients under treatment with monoclonal antibodies, especially when there is a new development of neurological signs or symptoms.Key words: JC virus - progressive multifocal leukoencephalopathy - recommendation - rituximab.
“进行性多灶性白质脑病”(PML)这一术语于1958年首次被用于描述淋巴增殖性疾病患者中一种致命的中枢神经系统(CNS)脱髓鞘疾病。1971年,导致该疾病的病毒被分离出来,并命名为约翰·坎宁安病毒(JCV)。我们报告一例62岁患有慢性淋巴细胞白血病并伴有PML的男性罕见病例。在我们的工作中,我们讨论了诊断和治疗方面的挑战,并为预防PML的发生提供了建议。主要要点如下:1. 定期检查免疫球蛋白水平和CD4+:CD8+ T细胞比值,当记录到其降低时应考虑静脉注射免疫球蛋白。2. 在检查CD4+:CD8+ T细胞比值并确认无法提高免疫球蛋白水平时,我们必须权衡继续使用单克隆抗体治疗的可能益处与风险。3. 医生应对接受单克隆抗体治疗的患者发生PML保持高度怀疑指数,尤其是当出现新的神经体征或症状时。关键词:JC病毒 - 进行性多灶性白质脑病 - 建议 - 利妥昔单抗