与AIDS相关的原发性中枢神经系统淋巴瘤(AR-PCNSL)是一种定义AIDS的疾病,通常在CD4计数小于50个细胞/μl时发生。在高活性抗逆转录病毒疗法(HAART)时代,该疾病的发生频率已大大减少。如果不治疗,预后较差,快速进展会导致2-3个月内死亡。
一名65岁男性因步态障碍接受医疗救治,体重减轻和轻微的左侧偏瘫。人类免疫缺陷病毒感染被诊断为初始CD4计数为116细胞/μl,病毒载量为260,000拷贝/ml。大脑的磁共振成像显示三个脑部病变,涉及右额叶和左顶叶,活检导致AR-PCNSL的诊断。全脑放疗(WBRT)开始HAART,患者拒绝全身化疗。由于性能状态不佳,他被转移到姑息治疗。根据HAART,他慢慢恢复,CD4计数恢复正常,病毒载量检测不到。医学影像学显示脑部病变完全缓解(CR)。在3年的随访中,患者仍处于CR状态,但表现为轻度神经认知功能障碍,可能继发于WBRT。
如今,治疗模式与基于全身化疗(主要是大剂量静脉注射甲氨蝶呤和类固醇)联合HAART的免疫活性人群中原发性中枢神经系统淋巴瘤的治疗模式相似.由于晚期神经毒性作用,WBRT的作用值得怀疑。
AIDS-related primary central nervous system lymphoma (AR-PCNSL) is an AIDS-defining disease that usually occurs when the CD4 count is less than 50 cells/μl. The frequency of the disease has substantially decreased in the era of highly active antiretroviral therapy (HAART). Prognosis is poor with rapid progression leading to death within 2-3 months if left untreated.
A 65 years old male presented to medical attention with gait disturbance, weight loss and slight left-sided hemiparesis. Human immunodeficiency virus infection was diagnosed with an initial CD4 count of 116 cells/µl and a viral load of 260,000 copies/ml. Magnetic resonance imaging of the brain revealed three brain lesions involving the right frontal lobe and the left parietal lobe, which on biopsy led to a diagnosis of AR-PCNSL. HAART was initiated with whole-brain radiotherapy (WBRT), and the patient declined systemic chemotherapy. Due to poor performance status, he was transferred to palliative care. Under HAART, he slowly recovered with normalization of CD4 count and undetectable viral load. Medical imaging showed complete remission (CR) of the brain lesions. At 3-year follow-up, the patient remains in CR, but presented mild neurocognitive dysfunction possibly secondary to WBRT.
Nowadays, treatment paradigm parallels that of primary central nervous system lymphoma in the immunocompetent population based on systemic chemotherapy (primarily high-dose intravenous methotrexate and steroids) in association with HAART. The role of WBRT is questionable because of late neurotoxic effects.