论文部分内容阅读
目的探讨首发或原发于淋巴结内的鼻型NK/T细胞淋巴瘤(NKTCL)的临床病理学特征,增强对此少见疾病的认识。方法对1例首发于淋巴结内的鼻型NKTCL的临床病理学特征、免疫表型及基因重排进行分析研究,并复习相关文献,综合分析首发于淋巴结内的鼻型NKTCL的临床及病理特征。结果患者三系细胞间断性减少3年伴低热,住院10天后发现颌下淋巴结肿大。活检显示淋巴结结构破坏、消失,伴大量血管纤维素样坏死,中等~大的异型淋巴样细胞弥漫分布,核仁较小或不明显,核分裂多见。免疫组化:肿瘤细胞胞质型CD3、CD30、TIA-1、CD4、CD8、CD43和EBER(+),MPO、CD20、CD79a、Pax-5、bcl-2、bcl-6、CD10、CD56、CD2、CD5、CD7、CD68和ALK(-)。PCR技术未检测到Ig H及TCRγ、β、δ基因重排。患者化疗及骨髓移植无效,5个月后死亡。结论首发于淋巴结内的NKTCL在组织形态学及免疫表型方面均与鼻腔或结外鼻型NKTCL相似,该病进展迅速,可能是结外鼻型NKTCL的亚型。
Objective To investigate the clinicopathological features of nasal NK / T cell lymphoma (NKTCL) in primary or primary lymph nodes and to enhance the understanding of this rare disease. Methods The clinical and pathological features, immunophenotypes and gene rearrangements of nasal type NKTCL, which originated in the lymph nodes, were analyzed and reviewed. Relevant literatures were reviewed, and the clinical and pathological features of nasal NKTCL in lymph nodes were analyzed. Results The patient’s triad cells decreased intermittently for 3 years with fever, and found submandibular lymph nodes 10 days after hospitalization. Biopsy showed the destruction of the lymph node structure, disappeared, with a large number of vascular fibrosis, moderate to large diffuse distribution of atypical lymphoid cells, small or not obvious nucleoli, mitotic more common. Immunohistochemistry: Cytosolic CD3, CD30, TIA-1, CD4, CD8, CD43 and EBER (+), MPO, CD20, CD79a, Pax-5, bcl- CD2, CD5, CD7, CD68 and ALK (-). PCR did not detect Ig H and TCRγ, β, δ gene rearrangements. Patient chemotherapy and bone marrow transplantation invalid, died after 5 months. Conclusions NKTCL, which originated in the lymph nodes, is similar to nasal or extranodal nasal NKTCL in histomorphology and immunophenotype. The disease progresses rapidly and may be a subtype of extranodal nasal NKTCL.