CAJ | 학술논문

目的探讨小细胞性非特指外周T细胞淋巴瘤(PTCL,NOS)的临床病理与免疫表型及其病理诊断和鉴别诊断.方法对5例小细胞性PTCL,NOS进行临床病理回顾性研究和随访,免疫表型检测(SP和EnVision法),以及EBER原位杂交和T细胞受体(TCR)基因重排分析.结果 5例均为男性,平均年龄52.6岁.中位病程1个月.5例中3例为临床Ⅳ期,2例为临床Ⅲ期.4例有全身浅表淋巴结及脾脏肿大,1例有肝肿大.2例有浆膜腔积液.行骨髓检查的4例中,3例有肿瘤累及.1例有外周血自细胞总数和淋巴细胞分类计数升高.主要病理改变为淋巴结结构的破坏和单一形态的小淋巴细胞弥漫性浸润,4例可见少数大的异形细胞散在分布,2例见小血管增生现象.5例之肿瘤细胞均表达两种以上T细胞分化抗原和CD43,表达CD99(3/4),均不表达CD20、末端脱氧核苷酸转移酶、CD56和粒酶B.Ki-67指数为5%-15%.4例行TCR基因重排分析,均存在TCRy基因克隆性重排,1例检出TCRβ基因克隆性重排.EBER原位杂交检测均为阴性.获得3例随访资料,且患者均死亡,平均生存时间21.7个月.结论小细胞性PTCL,NOS少见,呈高临床分期,预后差,组织形态表现为惰性淋巴瘤.
목적 탐토소세포성비특지외주T세포림파류(PTCL,NOS)적림상병리여면역표형급기병리진단화감별진단.방법 대5례소세포성PTCL,NOS진행림상병리회고성연구화수방,면역표형검측(SP화EnVision법),이급EBER원위잡교화T세포수체(TCR)기인중배분석.결과 5례균위남성,평균년령52.6세.중위병정1개월.5례중3례위림상Ⅳ기,2례위림상Ⅲ기.4례유전신천표림파결급비장종대,1례유간종대.2례유장막강적액.행골수검사적4례중,3례유종류루급.1례유외주혈자세포총수화림파세포분류계수승고.주요병리개변위림파결결구적파배화단일형태적소림파세포미만성침윤,4례가견소수대적이형세포산재분포,2례견소혈관증생현상.5례지종류세포균표체량충이상T세포분화항원화CD43,표체CD99(3/4),균불표체CD20、말단탈양핵감산전이매、CD56화립매B.Ki-67지수위5%-15%.4례행TCR기인중배분석,균존재TCRy기인극륭성중배,1례검출TCRβ기인극륭성중배.EBER원위잡교검측균위음성.획득3례수방자료,차환자균사망,평균생존시간21.7개월.결론 소세포성PTCL,NOS소견,정고림상분기,예후차,조직형태표현위타성림파류.
Objective To study the clinicopathologic features and differential diagnosis of small cell variant of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS).Methods The clinicopathologic features of 5 cases of small cell variant of PTCL, NOS were retrospectively reviewed, with immunohistochemical study, T-cell receptor(TCR) gene reatrangement analysis and evaluation for Epstein-Barr virus (EBV) status. Results All the 5 patients were males. The mean age was 52. 6 years. The median duration before diagnosis was 1 month. Clinically, 3 patients presented in stage Ⅳ and 2 in stage Ⅲ. Four of them had generalized lymphadenopathy and splenomngaly. Hepatomegaly and massive effusion were found in 1 and 2 cases, respectively. Marrow involvement was detected in 3 of the 4 patients with bone marrow biopsy performed and one of them also accompanied by lymphocytesis. Histologically, the involved lymph nodes showed partial or complete effacement of nodal architecture and replacement by a monomorphous population of small lymphoid cells. Scanty large lymphoid cells were also identified in 4 cases. Increase in number of blood vessels was noticed in two of them as well. Immunohistochemically, the lymphoma cells in all cases expressed two or more of the T-cell markers and CD43. The staining for CD20, TdT, CD56 and granzyme B was negative. CD99 expression was noted in 3 of the 4 cases. The Ki-67 index ranged from 5% to 15%. Clonal TCRy gene rearrangement was detected in the 4 cases studied and one of them also showed TCRβ gene rearrangement, In-situ hybridization for EBV-encoded RNA was negative in the 4 cases studied.Follow up information was available in 3 of the 5 cases. All of the 3 patients died of the disease, with an average survival of 21.7 months. Conclusion Small cell variant of PTCL, NOS represents a rare disease entity which often presents in advanced tumor stage and carries a poor prognosis.