肠病性T细胞淋巴瘤临床病理特征分析

目的:探讨肠病性T细胞淋巴瘤(enteropathic T-cell lymphoma,ETCL)临床病理特点、免疫表型及分子遗传学特征。方法:对1例ETCL的临床、病理组织学、免疫表型及免疫球蛋白重链和T细胞受体(TCR)基因克隆性重排进行观察并复习相关文献。结果:眼观部分回肠及右半结肠切除标本:回肠、结肠节段性溃疡。镜检回肠浅表溃疡病变累及黏膜层和黏膜下层,深度溃疡病变累及全层,肠壁各层在炎症背景下出现异常淋巴细胞浸润。结肠病变与回肠相同。免疫表型见瘤细胞呈CD43+,CD45RO+,CD56+,TIA1+,GB+,PF+,CD3-,CD3(单),CD30-,EMA-,CD20-,CD79a-。TCRγ重排:JVⅠ(+)JVⅡ(+)。EBER原位杂交检测(-)。结论:ETCL多发生于青年男性,临床症状多无特异性,病程发展迅猛,预后极差。病变的肠管表现为多发性溃疡,TCRγ克隆性重排。

Type of enteropathic T-cell lymphoma:a case report and review of the literatures

Objective:To investigate the clinicopathological features,immunophenotype and molecular genetics of enteropathic T-cell lymphoma(ETCL).Methods:The histopathological appearance,the immunophenotype,immunoglobulin heavy chain,the gene clonality rearrangement of T-cell receptor(TCR) of a ETCL cases were observed,and relevant references were reviewed.Results:Segmental ulcers were found in ileum and right colon.Superficial ulcer involving the ileal mucosa and submucosa,the deeper ulcerative lesions invasing the full thickness and abnormal lymphocytes encroached all layers of the intestinal wall in the context of inflammation of ileum and colon were observed under microscope.According to immunophenotype,the immunophenotype of CD43+,CD45RO+,CD56+,TIA1+,GB+ and PF+ of the tumor cells presented postive results,but the expression of CD3-,CD3(monoclone) CD30-,EMA-,CD20-and CD79a were negative.According to TCR-γ gene rearrangement,JVⅠ and JVⅡshowed positive,and the result of hybridization in situ of EBER was negative.Conclusions:ETCL occurs most in young men with no specific symptoms.Its progresses is rapidly and the result of prognosis is poor.The multiple ulcer in intestinal canal and TCR-γ gene rearrangement are its main characteristic.