Zhang Xiao, Liu Xin-Shu, Zhao Chan, Lai Ya-Min, Zhang Mei-Fen
Department of Ophthalmology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China.
Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China.
BMC Ophthalmol. 2017 Jun 23;17(1):99. doi: 10.1186/s12886-017-0494-3.
Intraocular involvement of systemic T-cell lymphomas are uncommon and have been broadly regarded as markers of poor prognosis. We reported two cases of uveitis patients finally diagnosed as systemic T cell lymphoma.
Case one is a 19-year-old female presented with fever and liver dysfunction, and was diagnosed as EBV-associated chronic active hepatitis. Fourteen months later, she suffered from recurrent granulomatous anterior uveitis in both eyes, which failed to respond to steroid and immunosuppressant therapy. A mass on the left side of pharynx was found and the final diagnosis was pharynx T cell non-Hodgkin's lymphoma. After 13 cycles of chemotherapy, her systematic symptoms and uveitis relieved a lot, and eye condition is stable after cataract surgery. Case two is a 37-year-old male complaining bilateral blurred vision and recurrent abdominal pain. Panuveitis was diagnosed and anterior inflammation did not release after topical steroid. During the following days, the patient complained intermittent abdominal pain and fever, with rapidly progressive bilateral visual decrease. Final diagnosis was gallbladder type II enteropathy-associated T-cell lymphoma. The patient died of multiple organ failure 4 days after operation that was only 26 days after presenting to our hospital.
Ocular manifestations as first signs of systemic T cell lymphoma were rare. Diagnosis of lymphoma has to be suspected when patients have systemic manifestations including fever, fatigue, abdominal pain, EBV-associated liver disease, et al., and uveitis fails to respond to steroid therapy.
系统性T细胞淋巴瘤的眼内受累并不常见，并且一直被广泛认为是预后不良的标志。我们报告了两例最终被诊断为系统性T细胞淋巴瘤的葡萄膜炎患者。
病例一为一名19岁女性，出现发热和肝功能障碍，被诊断为EB病毒相关的慢性活动性肝炎。14个月后，她双眼反复出现肉芽肿性前葡萄膜炎，对类固醇和免疫抑制剂治疗无效。在咽部左侧发现一个肿块，最终诊断为咽部T细胞非霍奇金淋巴瘤。经过13个周期的化疗，她的全身症状和葡萄膜炎有了很大缓解，白内障手术后眼部情况稳定。病例二为一名37岁男性，主诉双眼视力模糊和反复腹痛。诊断为全葡萄膜炎，局部使用类固醇后前炎症未缓解。在接下来的几天里，患者主诉间歇性腹痛和发热，双侧视力迅速进行性下降。最终诊断为胆囊II型肠病相关T细胞淋巴瘤。患者在我院就诊仅26天后，术后4天死于多器官功能衰竭。
眼部表现作为系统性T细胞淋巴瘤的首发症状很罕见。当患者出现包括发热、疲劳、腹痛、EB病毒相关肝病等全身表现，且葡萄膜炎对类固醇治疗无反应时，必须怀疑淋巴瘤的诊断。
