摘要
目的探讨淋巴结鼻型血管内NK/T细胞淋巴瘤的临床病理特征、诊断与鉴别诊断、治疗及预后,增强对此少见疾病的认识。方法回顾性分析1例淋巴结鼻型血管内NK/T细胞淋巴瘤患者的临床、组织病理形态、免疫表型及基因重排资料并进行分析研究,同时复习相关文献,综合分析淋巴结鼻型血管内NK/T细胞淋巴瘤的临床病理特征。结果光镜下淋巴结结构破坏,局部可见纤维素样退变坏死,可见片状中等-偏大体积的异型淋巴样细胞,肿瘤细胞胞质比较丰富淡染,细胞核圆形、卵圆形或不规则,染色质细颗粒状,可见核仁和核分裂象,部分肿瘤细胞团中央可见纤维素样退变坏死物。免疫组化:肿瘤细胞LCA、CD3ε、CD7、CD56、TIA-1和粒酶B(+),CD2、CD4、CD5、CD8、CD20、PAX-5、AE1/AE3、HMB45、melan-A、CD30和ALK均(-),CD31、CD34显示肿瘤细胞位于血管内,Ki-67阳性率>90%。EBER分子原位杂交>90%(+);PCR法进行Ig H、Κ、Λ和TCRγ、β、δ基因克隆性重排检测均为(-)。结论淋巴结鼻型血管内NK/T细胞淋巴瘤十分少见,临床和病理需要与转移癌、恶性黑色素瘤、转移性生殖细胞肿瘤、血管内大B细胞性淋巴瘤、血管内间变性大细胞淋巴瘤等病变鉴别,因此掌握其临床病理特征对诊断与鉴别诊断、治疗方案的选择及预后判断等均具有重要意义。
Objective To investigate the clinical and pathological features, diagnosis and differential diagnosis,treatment and prognosis of intranodal intravascular NK/T-cell lymphoma,nasal type. Methods Retrospective analysis of the clinical data was conducted in one case of intranodal intravascular NK/T-cell lymphoma, nasal type; the histopathological morphology,immunohistochemical staining,in situ hybridization staining and gene rearrangements were analyzed with review of relevant literatures. Results Under light microscope,the lymph node structure was destructed,the lymphatic sinus were disappeared,medium to large-sized atypical lymphoid cells were observed in the vascular volume;fibrinoid exudates were visible in the vascular cavity or around the vessels. The neoplastic cells had abundant light pink cytoplasm,the nuclei were round,oval or irregular with deep stained chromatin; nuclei and mitosis were visible.Immunohistochemical staining showed that the neoplastic cells were positive for LCA,CD3,CD7,CD56,TIA-1 and granzyme B,but CD2,CD4,CD5,CD8,CD20,Pax-5,CD30,ALK,AE1/AE3,HMB45 and Melan A were all negative; CD34 and CD31 staining showed that all the neoplastic cells were located endovascular space; Ki-67-positive rate was more than 90%. EBER in situ hybridization showed that above 90% of the nuclei were positive,and clonal Ig H,Κ Λand TCR γ,β,δ gene rearrangements were not found by polymerase chain reaction（ PCR）. Conclusions Intranodal intravascular NK/T-cell lymphoma,nasal type,is a rare type of intravascular lymphoma,and it needs to be distinguished from IVLBCL,IVALCL,metastatic carcinoma and others. Therefore,grasping the clinical and pathological features of intranodal intravascular NK/T-cell lymphoma, nasal type, will play an important role in the diagnosis, differential diagnosis,treatment and prognosis of this unique rare disease.
作者
张立英
祁晓莉
皋岚湘
秦云
任平
李悦
杜睿
卢晓红
刘洪波
ZHANG Li-ying;QI Xiao-li;GAO Lcm-xiang;QIN Yun;REN Ping;LI Yue;DU Rui;LU Xiao-hong;LIU Hong-bo(Department of Pathology,Daxing Teching Hospital of Capital Medical University,Beijing 102600,China;Department of Pathology,PLA Army General Hospital,Beijing 100700,China)
出处
《诊断病理学杂志》
2018年第7期515-520,共6页
Chinese Journal of Diagnostic Pathology
