Kirn Thomas J, Levy Norman B, Gosselin J J Benoit, Rosen William J, Zegans Michael E
Department of Microbiology and Immunology, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03756, USA.
Cornea. 2007 Oct;26(9):1147-9. doi: 10.1097/ICO.0b013e31812dfa88.
To report an unusual case of ocular peripheral T-cell lymphoma presenting as sclerouveitis.
A 53-year-old woman presented with painful, unilateral sclerouveitis and was initially treated with antivirals and corticosteroids for what was presumed to be isolated ocular HSV infection with vasculitis. When she failed to improve the conjunctiva was biopsied and characterized by immunohistochemical and molecular methods. The literature regarding conjunctival T-cell lymphomas as well as lymphomas mimicking scleritis is reviewed.
Biopsies of a scleral/conjunctival nodule from the right eye were obtained on 2 occasions, the second of which revealed the presence of intermediate to large size lymphocytes that were CD3, T-cell intracellular antigen 1 and Granzyme B positive but CD56 and Epstein Barr virus negative. Polymerase chain reaction showed clonal T-cell receptor gamma rearrangements in DNA samples isolated from biopsy tissue. Another biopsy was obtained from a mucosal lesion in the oropharynx, which was shown to contain an identical T-cell receptor gamma rearrangement. These results led to the diagnosis of peripheral T-cell lymphoma. Conjunctival T-cell lymphomas have been reported in two other patients both of which also had involvement of upper airway structures. Five other reports of lymphoma mimicking scleritis are discussed.
Peripheral T-cell lymphoma should be considered in the differential diagnosis of patients who present with sclerouveitis of unknown etiology unresponsive to conventional therapy. If a conjunctival T-cell lymphoma is identified a systemic evaluation for lymphoma with particular attention to the upper airway is justified.
报告一例表现为巩膜外层炎的眼部外周T细胞淋巴瘤罕见病例。
一名53岁女性因单侧巩膜外层炎伴疼痛就诊，最初因疑似孤立性眼部单纯疱疹病毒感染伴血管炎而接受抗病毒药物和皮质类固醇治疗。当病情无改善时，对结膜进行活检，并采用免疫组织化学和分子方法进行特征分析。回顾了有关结膜T细胞淋巴瘤以及模仿巩膜炎的淋巴瘤的文献。
两次获取右眼巩膜/结膜结节的活检组织，第二次活检显示存在中等大小至大尺寸淋巴细胞，这些细胞CD3、T细胞胞内抗原1和颗粒酶B呈阳性，但CD56和爱泼斯坦-巴尔病毒呈阴性。聚合酶链反应显示从活检组织分离的DNA样本中存在克隆性T细胞受体γ重排。从口咽部黏膜病变处获取另一活检组织，显示含有相同的T细胞受体γ重排。这些结果导致诊断为外周T细胞淋巴瘤。另外两名患者也报告了结膜T细胞淋巴瘤，他们的上呼吸道结构也均有受累。还讨论了其他五例模仿巩膜炎的淋巴瘤报告。
对于病因不明且对传统治疗无反应的巩膜外层炎患者，鉴别诊断时应考虑外周T细胞淋巴瘤。如果确诊为结膜T细胞淋巴瘤，对淋巴瘤进行全身评估，尤其关注上呼吸道是合理的。
