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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 a case of misdiagnosis of intestinal nasal type NK / T cell lymphoma in children in order to enhance understanding of the disease among pediatric clinicians and pathologists. Methods Clinicopathological analysis combined with immunohistochemical staining, Epstein-Barr virus in situ hybridization and T cell receptor gene rearrangement PCR detection analysis confirmed. Results The first biopsy of the intestinal wall tissue of the lesion was diagnosed as chronic granulomatous inflammatory changes. 11 days later, the intestinal wall tissue and mesenteric lymph node biopsy were diagnosed as non-Hodgkin’s lymphoma derived from small, non-cleaved T cells. Re-reconstructed film found that the two specimens of the full-thickness intestinal wall and mesenteric lymph nodes see a large number of tumor cell infiltration, tumor cells of medium size, atypia obvious central vascular invasion and vascular necrosis, inflammatory background. Tumor cells cCD3, CD8, CD56, TIA-1 and granzymeB (+), CD4 and CD45RO (-), LMP (-) and EBER (+); Finally confirmed as intestinal NK / T cell lymphoma, nasal type. Conclusion Children with intestinal NK / T cell lymphoma rare, high malignancy, poor prognosis, the nature of the tumor is easily covered by the inflammatory background, the diagnosis depends on conventional histopathology combined with immunohistochemistry and molecular pathology.