摘要
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％）、CDl0（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。
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.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2014年第1期24-28,共5页
Chinese Journal of Hematology
基金
蚌埠医学院科研发展基金重点项目（BYKF12A08）
关键词
淋巴瘤
T细胞
外周
病理学
临床
免疫组织化学
诊断
鉴别
Lymphoma, T-cell, peripheral
Pathology, clinical
Immunohistochemistry
Diagnosis, differential
