Li Nan, Wang Yi-Zhuo, Zhang Yi, Zhang Wei-Ling, Zhou Yan, Huang Dong-Sheng
Pediatric Department, Beijing Tongren Hospital Affiliated to Capital Medical University, Beijing 100176, China.
World J Clin Cases. 2020 Aug 6;8(15):3284-3290. doi: 10.12998/wjcc.v8.i15.3284.
Because of atypical clinical symptoms, lymphoma is easily confused with infectious diseases. Extranodal nasal-type natural killer/T-cell lymphoma (NKTL) is more common, and there are few cases of eyelid site onset and intracranial infiltration, which increases the difficulty of diagnosis. This disease usually has a very poor prognosis and there are few reports of recovery.
A 3-year-old boy was admitted to our hospital due to an initial misdiagnosis of "eyelid cellulitis" and failed antibiotic treatment. He was characterized by fever, right eyeball bulging, convulsions, and abnormal liver function. His blood Epstein-Barr virus (EBV) DNA was positive (8.798 × 10 copies/mL), and remained positive for about half a year. The cranial imaging examination suggested a space-occupying lesion in the right eyelid, with the right temporal lobe and meninges involved. The boy underwent ocular mass resection. The pathological diagnosis was NKTL. He was diagnosed as having NKTL with intracranial infiltration, combined with chronic active EBV infection (CAEBV). Then he underwent systemic chemotherapy and intrathecal injection. The boy suffered from abnormal blood coagulation, oral mucositis, diarrhea, liver damage, and severe bone marrow suppression but survived. Finally, the tumor was completely relieved and his blood EBV-DNA level turned negative. The current follow-up has been more than 2 years and his condition is stable.
This case suggests that chemotherapy combined with intrathecal injection may have a good effect on intracranial infiltrating lymphoma and CAEBV, which deserves further study and discussion.
由于临床症状不典型，淋巴瘤容易与传染病混淆。结外鼻型自然杀伤/T细胞淋巴瘤（NKTL）较为常见，眼睑部位发病并伴有颅内浸润的病例较少，这增加了诊断难度。这种疾病通常预后很差，康复的报道也很少。
一名3岁男孩因最初被误诊为“眼睑蜂窝织炎”且抗生素治疗无效而入住我院。他的特点是发热、右眼球突出、抽搐和肝功能异常。他的血液中爱泼斯坦-巴尔病毒（EBV）DNA呈阳性（8.798×10拷贝/mL），并持续阳性约半年。头颅影像学检查提示右眼睑占位性病变，累及右侧颞叶和脑膜。该男孩接受了眼部肿物切除术。病理诊断为NKTL。他被诊断为颅内浸润性NKTL，合并慢性活动性EBV感染（CAEBV）。随后他接受了全身化疗和鞘内注射。该男孩出现了凝血异常、口腔黏膜炎、腹泻、肝损伤和严重的骨髓抑制，但存活了下来。最终，肿瘤完全缓解，他的血液EBV-DNA水平转为阴性。目前随访已超过2年，他的病情稳定。
该病例表明化疗联合鞘内注射可能对颅内浸润性淋巴瘤和CAEBV有较好的疗效，值得进一步研究和探讨。
