儿童肠道鼻型NK/T细胞淋巴瘤误诊病例分析  

Pediatric intestinal NK /T-cell lymphoma,nasal-type:an analysis of misdaignosed case

作　　者：周春菊[1] 宫丽平[2] 王琳[1] 张蕊[3] 杜新宇[1] 刘靖[1] 

机构地区：[1]附属北京儿童医院病理科,北京100045 [2]基础医学院病理学系,北京100054 [3]附属北京儿童医院血液科,北京100045

出　　处：《诊断病理学杂志》2010年第3期186-189,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的报道1例儿童肠道鼻型NK/T细胞淋巴瘤误诊病例,以加强儿科临床医师和病理医师对此病的认识。方法通过临床病理分析结合免疫组化染色、EB病毒原位杂交及T细胞受体基因重排的PCR检测分析确诊。结果病变肠壁组织初次活检诊断为慢性肉芽肿炎性改变,11天后再次肠壁组织及肠系膜淋巴结活检诊断为小无裂型T细胞来源的非霍奇金淋巴瘤。重新复片发现,两次检材肠壁全层及肠系膜淋巴结见大量肿瘤细胞浸润,瘤细胞中等大小、异型性明显,血管中心性浸润和血管坏死,炎性背景明显。瘤细胞cCD3、CD8、CD56、TIA-1和granzymeB(+),CD4和CD45RO(-),LMP(-),EBER(+);未检测到TCR基因的克隆性重排。最后确诊为肠道NK/T细胞淋巴瘤,鼻型。结论儿童肠道NK/T细胞淋巴瘤少见,恶性度高,预后差,肿瘤本质易被炎性背景掩盖,诊断有赖于常规组织病理结合免疫组化和分子病理技术。Objective To report one misdiagnosed case of pediatric intestinal NK/T-cell lymphoma, nasal-type to draw the attention of physicians and pathologists. Methods The clinicopathologic analysis, immunohistochemistry, in situ hybridization for EBER and PCR for TCR-gene rearrangement were used in this study. Results It was diagnosed as a chronic granulomatous infammatory changes in the first biopsy;ll days later the intestinal wall and mesenteric lymph node biopsy was again diagnosed as a small non-cleaved T cell non-Hodgkin＇ s lymphoma. Review of the sections found that a large number of tumor cell infiltration were observed in intestinal wall and mesenteric lymph nodes in both biopsies. The medium-sized tumor cells showed marked atypia, angiocentric infiltration with vascular necrosis and marked inflammatory background. The tumor cells were positive for cCD3, CD8, CD56, TIA-1 and granzymeB, and negitive for CD4 and CD45RO; EBER was detected by in situ hybridization; No gene rearrangement of TCRs was found. Finally This case was diagnosed as intestinal nasal-type NK/T cell lymphoma. Conclusion Pediatric intestinal NK/T-cell lymphoma, nasal type, is a high-grade malignancy with poor prognosis. The diagnosis depends on the combination of routine histopathological and molecular pathologic techniques.

关 键 词：淋巴瘤 NK/T细胞 肠道 儿童 

分 类 号：R739.62[医药卫生—肿瘤]