皮下脂膜炎性T细胞淋巴瘤1例报告及文献复习  

SUBCUTANEOUS PANNICULITIC T-CELL LYMPHOMA:REPORT OF A CASE AND REVIEW OF THE LITERATURE

作　　者：许瑞莲 陈伟[1] 吴铭 徐肇明 

机构地区：[1]暨南大学医学院第二附属医院,深圳市人民医院,广东深圳518020

出　　处：《白血病．淋巴瘤》2003年第5期282-284,共3页Journal of Leukemia & Lymphoma

摘　　要：目的 :提高对皮下脂膜炎性 T细胞淋巴瘤 (SPTCL)的临床和病理特点的认识。方法 :报告 1例侵袭性 SPTCL 病例 ,并进行文献复习。结果 :皮损为多发或单发的红斑结节。病变主要侵犯皮下脂肪组织 ,在脂膜炎背景上 ,既有肿瘤性多形性 T细胞浸润 ,又有反应性组织细胞增多 ,常伴噬血细胞综合征 (HPS)。瘤细胞表达 CD4 5、CD4 5RO、CD3、CD2 、CD8、CD2 5、CD4 3、TIA、TCR,不表达CD4 、CD1 9、CD2 0 。80 %病例有 TCR基因重排 ,其中以γ链、β链检出频率较高。结论 :SPTCL是 CD+ 8T细胞克隆性疾病、原发于皮下脂肪组织的外周Objective:In order to raise understanding of subcutaneous panniculitic T cell lymphoma(SPTCL).Methods:An unusual case of SPTCL was reported and the characteristics of SPTCL were discussed with literature review.Results:Multiple or single erythematous nodules or plaques were charecteristic,and the lower extremities were the most frequent site of cutaneous involvement.Histologically,the neoplastic cells involed the subcutaneous fat tissue in a panniculitis like pattern,activated macrophages,and were frequently complicated by the hemophagocytic syndrome(HPS).The neoplastic cells immunostained positive for CD 45 、CD 45RO 、CD 3、CD 2、CD 8、CD 25 、CD 43 、TIA、TCR,but negative for CD 4、CD 19 、CD 20 .The clonal rearrangement of TCR gene was detected in 80 % of the cases,the γ chain and β chain occurred more frequently than α chain and δ chain.Conclusion:SPTCL is chonal expanded disease of CD + 8 T cell origin and considered to be a new subtype of peripheral T cell lymphoma.

关 键 词：皮肤脂膜炎T细胞淋巴瘤 TCR基因重排 病理特点 临床诊断 

分 类 号：R739.5[医药卫生—肿瘤]