摘要
目的探讨眼眶自然杀伤（NK）-T细胞淋巴瘤患者的临床表现、诊断和治疗方法。方法为回顾性系列病例研究。收集1982年至2004年间7例住院手术、经病理检查证实为眼眶NK-T细胞淋巴瘤患者的临床和病理检查资料，包括手术方式、治疗方法、肿瘤病理标本的HE、免疫组织化学染色和分子生物学检查结果。结果7例患者均表现为眼球突出、活动受限或固定，视力下降或失明，内眦部和眼睑皮肤红肿、溃烂和腔洞形成，CT检查可见眶内侧有边界欠清、密度不均的肿块。病理活检可见肿瘤内有大量淋巴细胞浸润和坏死组织，CD3、CD45、RO、CD57及Ki-67免疫组织化学染色阳性。2例患者T细胞受体基因重排呈阴性表达。而EB病毒的mRNA检查呈阳性。眶内肿瘤切除、局部放射及全身化学治疗效果均欠佳。结论眼眶NK-T细胞淋巴瘤少见，其特点是发病急，病变进展快且破坏性大，预后差。最后确诊需借助于组织病理学、免疫组织化学或分子生物学检查结果。（中华眼科杂志，2008，44：42-45）
Objective To investigate clinical manifestation, diagnosis and treatment of orbital natural killer（ NK） -T cell lymphoma. Methods It was a retrospective case series. Seven orbital NK-T cell lymphoma patients confirmed by surgical biopsies were collected during the past 22 years, We reviewed the records, surgical and treatment procedures. Surgical specimens were studied with HE staining, staining and molecular biological analysis. Results These patients had proptosis, eye motive inhibition or fixation and visual acuity was decreased or even without light perception. Skin of inner canthus and eyelids appeared red and swollen, with ulceration and cavity formation. CT scan revealed that the tumor showed uneven density and an unclear border. Tremendous lymphocyte infiltration and tissue necrosis in the tumor were observed in the biopsy tissue. LCA, CD45RO and CD57 staining revealed positive results. Clonal T-cell-receptor gene rearrangements of two patients showed negative results and the Epstein-Barr virus was detected. Conclusions Orbital NK-T cell lymphoma is a rare disease. The characteristics of this disease include a highly aggressive clinical course, severe destruction and a poor prognosis. The final diagnosis depends on HE staining, immunohistochemical staining and molecular biological examination. （Chin J Opbtbalmol , 2008,44：42-45）
出处
《中华眼科杂志》
CAS
CSCD
北大核心
2008年第1期42-45,共4页
Chinese Journal of Ophthalmology
关键词
眶肿瘤
淋巴瘤
T细胞
免疫组织化学
基因重排
T淋巴细胞
Orbital neoplasms
Lymphoma, T-cell
Immunohistochemistry
Gene rearrangement, T-lymphocyte
