首发于淋巴结内鼻型NK/T细胞淋巴瘤临床病理特征  

Primary nodal NK/T-cell lymphoma of nasal type: analyses of clinicopathological features

作　　者：陈少红[1] 杨静[1] 许进[1] 伍丽琼[1] 王小拍[1] 杜洪[1] 

机构地区：[1]广州市第一人民医院病理科,广州510180

出　　处：《诊断病理学杂志》2016年第6期433-436,共4页Chinese Journal of Diagnostic Pathology

摘　　要：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 primary nodal NK / T-cell lymphoma of nasal type（ NKTCL）. Methods Clinicopathological features,immunohistochemical characteristics and gene rearrangements were analyzed in a case of primary nodal NKTCL,and relevant literatures were reviewed. Results A 23-year-old woman presented as recurrent pancytopenia,low fever for 3 years and enlarged submandibular lymph node was found in our hospital after 10 days. Biopsy demonstrated lymph node structure was infitrated by numerous atypical medium to larger lymphoid cells with angiodestruction and fibrinoid necrosis. Mitotic figures were numerous. Atypical cells with little or inconspicuous nucleoli typically expressed cytoplastic CD3,CD30,TIA-1,CD4,CD8,CD43 and EBER,but not MPO,CD20,CD79 a,Pax-5,bcl-2,bcl-6,CD10,CD56,CD2,CD5,CD7,CD68 and ALK. Ig H and TCRγ,β,δ gene rearrangements were not found by polymerase chain reaction（ PCR）. Five months later,the patient died with no effect of chemotherapy and bone marrow transplantation. Conclusion Histopathologic features of primary nodal NK / T-cell lymphoma of nasal type are similar to nasal or extranasal NK / T-cell lymphoma,both of which are rapid progressive diseases. Primary nodal NK / Tcell lymphoma of nasal type may be a subtype of extranodal NK / T-cell lymphoma,nasal type.

关 键 词：原发性 淋巴结 鼻型NK/T细胞淋巴瘤 临床病理学 

分 类 号：R733.1[医药卫生—肿瘤]