原发EB病毒阳性结内T/NK细胞淋巴瘤的临床病理学分析

Clinicopathological features of primary EB virus positive nodal T/NK cell lymphoma

目的探讨原发EB病毒(EBV)阳性结内T/NK细胞淋巴瘤(TNKL)的临床病理特征。方法收集郑州大学第一附属医院2015年11月至2019年5月经病理确诊为EBV阳性结内TNKL的病例,应用免疫组织化学、原位杂交及聚合酶链反应(PCR)分析其病理学特点,并收集临床随访资料。结果(1)7例EBV阳性结内TNKL病例中男性5例,女性2例,年龄范围41~75岁,中位年龄54岁。所有病例原发部位均位于淋巴结。B症状常见(5/7)但骨髓累及少见(1/7),6例处于Ⅲ/Ⅳ期。(2)6例提示为T细胞起源的病例均表现为单形性肿瘤细胞形态,免疫表型CD56阴性,TCRαβ+/TCRγδ-,TCR基因重排单克隆性表达;只有1例提示为NK细胞起源的病例表现为多形性肿瘤细胞形态,免疫表型CD56阳性,TCRαβ-/TCRγδ-,TCR基因重排多克隆性表达。所有病例均表达细胞毒性标记,但CD4/CD8表达存在异质性。EBV感染模式均为弥漫阳性,阳性细胞数>100个/HPF。(3)7例中有6例化疗,1例未经治疗。随访时间3~48个月,5例死于本病。结论EBV阳性结内TNKL是一组好发于老年人原发于淋巴结内且具有侵袭性临床病程的淋巴瘤,起源于T/NK细胞且表达细胞毒性标记,应与其他EBV阳性的T/NK淋巴组织增生性疾病特别是结外NK/T细胞淋巴瘤进行鉴别。

Objective To investigate the clinicopathological features of primary Epstein-Barrvirus(EBV)positive nodal T/NK-cell lymphomas(EBV+nodal TNKL).Methods The clinicopathological features of 7 cases of EBV+nodal TNKL diagnosed between November 2015 and May 2019 at the First Affiliated Hospital of Zhengzhou University were analyzed using immunohistochemistry,PCR gene rearrangement and in situ hybridization.Follow-up data were also collected.Results There were 5 males and 2 females with a median age of 54 years(ranged from 41 to 75 years).All patients presented with multiple lymphadenopathies and common B symptoms(5/7)and at an advanced Ann Arbor stageⅢ/Ⅳ(6/7).Bone marrow involvementwas detected in 1 patient.Six cases of T-cell origin had monomorphic patterns,and the tumor cells showed CD56 negativity and TCRαβ+/TCRγδ-with T-cell clonality.One case of NK-cell origin had polymorphic pattern,and the tumor cells showed CD56 positivity and TCRαβ-/TCRγδ-without T-cell clonality.All cases were positive for the cytotoxic markers,but showed various CD4/CD8 expression.All 7 cases were diffusely positive for EBV(>100 cell/high power field).Six of the patients received chemotherapy,and 1 patient declined the treatments.During the follow-up period ranging from 3 to 48 months,5 of the 7 patients died of the disease.Conclusions EBV+nodal TNKL is a rare entity and is characterized by cytotoxic molecule expression,T/NK-cell derivation,and a predominance of nodal involvement at an advanced stage.It should be differentiated from other EBV+T/NK cell lymphoproliferative disorders,especially extranodal NK/T cell lymphoma.