CAJ | 학술논문

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.
목적 탐토안광자연살상(NK)-T세포림파류환자적림상표현、진단화치료방법.방법 위회고성계렬병례연구.수집1982년지2004년간7례주원수술、경병리검사증실위안광NK-T세포림파류환자적림상화병리검사자료,포괄수술방식、치료방법、종류병리표본적HE、면역조직화학염색화분자생물학검사결과.결과 7례환자균표현위안구돌출、활동수한혹고정,시력하강혹실명,내자부화안검피부홍종、궤란화강동형성,CT검사가견광내측유변계흠청、밀도불균적종괴.병리활검가견종류내유대량림파세포침윤화배사조직,CD3、CD45、RO、CD57급Ki-67면역조직화학염색양성.2례환자T세포수체기인중배정음성표체.이EB병독적mRNA검사정양성.광내종류절제、국부방사급전신화학치료효과균흠가.결론 안광NK-T세포림파류소견,기특점시발병급,병변진전쾌차파배성대,예후차.최후학진수차조우조직병이학、면역조직화학혹분자생물학검사결과.
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,immunohistochemical 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 immunohistochemical 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.