JC多瘤病毒(JCPyV)是一种全球分布的病毒,通常由免疫系统控制。在艾滋病患者中,潜伏性JCPyV感染可重新激活并发展为进行性多灶性白质脑病(PML).大约一半的PML患者在确诊后2年内死亡,然而在极少数情况下,这种疾病进展得更快,似乎不容易受到任何医疗行动的影响。在我们的诊所里,我们在艾滋病阶段的HIV阳性患者中观察到了两例此类病程。一入场,两名患者都有轻微的神经症状,如头晕,视力障碍,肌肉无力。两者的CD4淋巴细胞计数极低(7个细胞/μL,40细胞/μL)和高HIV-1病毒载量(VL)(50,324拷贝/ml,78,334份/毫升)。PML通过脑脊液(CSF)中JCPyVDNA的PCR以及临床和放射学特征得到证实。尽管接受了抗逆转录病毒(ARV)治疗与静脉内(IV)类固醇,在入院后的几周内,疾病进展迅速,神经系统表现加重.最终,2例患者均出现呼吸衰竭,并在出现神经系统症状后不到3个月内死亡.即使这种疾病的诅咒并不常见,这应该是对所有PML和艾滋病都可能致命的警告,并提醒医生甚至对无症状的患者提供检测。
The JC Polyomavirus (JCPyV) is a virus of global distribution and is usually kept under control by the immune system. In patients with AIDS, a latent JCPyV infection can reactivate and develop into progressive multifocal leukoencephalopathy (
PML). Around half of the patients with
PML die within 2 years since the diagnosis, yet in rare cases, the disease advances significantly quicker and seems to be insusceptible to any medical actions. In our clinic, we observed two cases of such course in HIV-positive patients in the AIDS stage. On admission, both patients had mild neurological symptoms such as dizziness, vision disturbances, and muscle weakness. Both had extremely low CD4 lymphocyte count (7 cells/μL, 40 cells/μL) and high HIV-1 viral load (VL) (50,324 copies/ml, 78,334 copies/ml).
PML was confirmed by PCR for JCPyV DNA in cerebrospinal fluid (CSF) coupled with clinical and radiological features. Despite receiving though antiretroviral (ARV) treatment paired with intra-venous (IV) steroids, the disease progressed rapidly with neurological manifestations exacerbating throughout the few weeks following the admission. Eventually, both patients developed respiratory failure and died within less than 3 months after the onset of the neurological symptoms. Even though such curse of the disease is not common, it should be a warning to all how deadly both
PML and AIDS can be and remind doctors to offer testing even to asymptomatic patients.