CAJ | 학술논문

目的探讨血管免疫母细胞性T细胞淋巴瘤(AITL)的临床病理特征.方法对64例AITL患者临床病理资料进行回顾性分析并增加免疫组化标记检测.结果 64例患者中,男35例,女29例,中位年龄59(25～84)岁.临床表现为进展期,出现淋巴结、肝脾肿大及全身症状.淋巴结病理组织细胞形态学检查结果显示淋巴结结构部分或全部消失,多形性细胞浸润,伴有明显的分支状高内皮小静脉和滤泡树突细胞增生,大部分患者出现单克隆T淋巴细胞异常增生.免疫组化检查结果显示瘤细胞表达趋化因子配体13(阳性率95.3％)、程序性死亡分子1(阳性率75.0％)、CD10(阳性率25.0％)、Bcl-6(阳性率40.0％)、CD2(阳性率96.0％)、CD3(阳性率95.0％)、CD4(阳性率84.0％)、CD5(阳性率73.0％),EBV编码的小RNA(阳性率39.5％),Ki-67(阳性率平均55.0％),常伴CD7、CD8表达减弱或丢失.结论 AITL起源于CD4+的滤泡辅助性T淋巴细胞,具有特殊的临床病理特征;病变局限者与外周T细胞淋巴瘤滤泡亚型在形态及免疫表型上可有重叠.
목적 탐토혈관면역모세포성T세포림파류(AITL)적림상병리특정.방법 대64례AITL환자림상병리자료진행회고성분석병증가면역조화표기검측.결과 64례환자중,남35례,녀29례,중위년령59(25～84)세.림상표현위진전기,출현림파결、간비종대급전신증상.림파결병리조직세포형태학검사결과현시림파결결구부분혹전부소실,다형성세포침윤,반유명현적분지상고내피소정맥화려포수돌세포증생,대부분환자출현단극륭T림파세포이상증생.면역조화검사결과현시류세포표체추화인자배체13(양성솔95.3％)、정서성사망분자1(양성솔75.0％)、CD10(양성솔25.0％)、Bcl-6(양성솔40.0％)、CD2(양성솔96.0％)、CD3(양성솔95.0％)、CD4(양성솔84.0％)、CD5(양성솔73.0％),EBV편마적소RNA(양성솔39.5％),Ki-67(양성솔평균55.0％),상반CD7、CD8표체감약혹주실.결론 AITL기원우CD4+적려포보조성T림파세포,구유특수적림상병리특정;병변국한자여외주T세포림파류려포아형재형태급면역표형상가유중첩.
Objective To explore the clinical and pathological characteristics of angioimmunoblastic T-cell lymphoma (AITL).Methods Sixty-four cases of AITL were retrospectively analyzed by histopathological and immunohistochemical methods.Results There were 35 men and 29 women,the median age was 59 years (range,25-84 ys).AITL typically presented with advanced stage,generalized lymphadenopathy,hepatosplenomegaly and systemic symptoms.Morphologically,the lymph nodes showed partial or total obliteration of the normal architecture by a polymorphic infiltration of lymphocytes,and by proliferation of follicular dendritic cells and that of high endothelial venules.Most cases contained a monoclonal T-cell population as well as clonal cytogenetic abnormalities.Immunophenotype analysis showed that neoplastic cells expressed the following markers:CXCL13(positive rate 95.3％),PD-1 (positive rate 75.0％),CD10 (positive rate 25.0％),Bcl-6 (positive rate 40.0％),CD2 (positive rate 96.0％),CD3 (positive rate 95.0％),CD4 (positive rate 84.0％),CD5 (positive rate 73.0％),EBER (positive rate 39.5％) and Ki-67 (average positive rate 55.0％),and frequently showed aberrant loss or reduced expression of CD7 and CD8.Conclusions The neoplastic cells of AITL showed features of CD4 + TFH,with peculiar clinical features.Peripheral T-cell lymphomas with a follicular growth pattern may show overlapping features with focal AITL.