Matsuda Hiroto, Hayashi Koichi, Meguro Mariko, Saruta Takao
Department of Internal Medicine, School of Medicine, Keio University, Tokyo, Japan.
Ther Apher Dial. 2006 Jun;10(3):291-5. doi: 10.1111/j.1744-9987.2006.00371.x.
We experienced a case manifesting progressive multifocal leukoencephalopathy (PML) in a hemodialytic patient with hepatitis C virus-induced liver cirrhosis and human T-cell lymphotropic virus type-1 (HTLV-1)-associated uveitis. A 57-year-old male patient had received chronic hemodialysis therapy for 10 years, during which he received multiple blood transfusions and HTLV-1-associated uveitis developed. He complained of visual disturbance and disorientation. Brain CT scan showed diffuse and multifocal low density areas in occipital and temporal lobes, with gray matter relatively spared. MRI imaging showed high intensity lesions in the same areas. Cerebrospinal fluid culture was negative, but using nested PCR, rearranged regulatory region of JC virus DNA was detected. His consciousness level gradually deteriorated and complete paraplegia developed. Seven months after admission, he died of pneumonia. An autopsy confirmed the diagnosis of PML. Notably, mononuclear cell infiltration, gliosis and demyelinating lesions but no nuclear inclusion bodies were observed in the thoracic cord, which suggested HTLV-1-associated myelopathy. Because JC virus is activated under immunocompromised conditions, precipitating factors in this case appear multifactorial; depressed immune system induced by chronic hemodialysis as well as blood-borne hepatitis C virus/HTLV-1 infection might contribute to the activation of dormant JC virus and the development of florid clinical manifestation of PML.
我们遇到了一例患有丙型肝炎病毒引起的肝硬化和人类嗜T淋巴细胞病毒1型(HTLV-1)相关性葡萄膜炎的血液透析患者出现进行性多灶性白质脑病(PML)的病例。一名57岁男性患者接受了10年的慢性血液透析治疗,在此期间他接受了多次输血并患上了HTLV-1相关性葡萄膜炎。他主诉视力障碍和定向障碍。脑部CT扫描显示枕叶和颞叶有弥漫性多灶性低密度区,灰质相对未受影响。MRI成像显示相同区域有高强度病变。脑脊液培养为阴性,但使用巢式PCR检测到了JC病毒DNA的重排调节区。他的意识水平逐渐恶化并发展为完全性截瘫。入院七个月后,他死于肺炎。尸检证实了PML的诊断。值得注意的是,在胸段脊髓观察到单核细胞浸润、胶质增生和脱髓鞘病变,但未观察到核内包涵体,这提示为HTLV-1相关性脊髓病。由于JC病毒在免疫功能低下的情况下被激活,该病例的诱发因素似乎是多因素的;慢性血液透析诱导的免疫系统抑制以及血源性丙型肝炎病毒/HTLV-1感染可能有助于激活潜伏的JC病毒并导致PML明显的临床表现。