Kato Seiichi, Asano Naoko, Miyata-Takata Tomoko, Takata Katsuyoshi, Elsayed Ahmed Ali, Satou Akira, Takahashi Emiko, Kinoshita Tomohiro, Nakamura Shigeo
*Department of Pathology and Laboratory Medicine, Nagoya University Hospital ∥Department of Hematology and Cell Therapy, Aichi Cancer Center, Nagoya †Department of Molecular Diagnosis, Nagano Prefectural Suzaka Hospital, Suzaka ‡Department of Pathology, Okayama University, Okayama §Department of Pathology, Aichi Medical University Hospital, Nagakute, Japan.
Am J Surg Pathol. 2015 Apr;39(4):462-71. doi: 10.1097/PAS.0000000000000323.
Among Epstein-Barr virus (EBV)-positive cytotoxic T/NK-cell lymphoma, there are only a few reports on the clinicopathologic features of patients with primary nodal presentation (nodal EBV cytotoxic T-cell lymphoma [CTL]). Here, we compared the clinicopathologic profiles of 39 patients with nodal EBV CTL with those of 27 cases of "extranasal" NK/T-cell lymphoma of nasal type (ENKTL), especially addressing their T-cell receptor (TCR) phenotype. Histologically, 22 of 39 nodal EBV CTL cases (56%) were unique in having centroblastoid appearance, which was contrasted with the lower incidence of this feature in ENKTL (15%, P=0.001). In contrast, pleomorphic appearance was more frequently seen in ENKTL than in nodal EBV CTL (67% vs. 23%, P=0.001). Thirty-three of 39 nodal EBV CTL cases (85%) were of T-cell lineage on the basis of TCR expression and/or TCRγ gene rearrangement; in detail, 18 cases (46%) were TCRβ positive (αβ T), 5 (13%) were TCRγ and/or δ positive (γδ T), and 10 (26%) were TCR-silent type with clonal TCRγ gene rearrangement but no expression of TCRβ, γ, or δ. These results were clearly contrasted by a lower incidence of T-cell lineage in ENKTL (7 cases, 26%, P<0.001). Notably, the survival time of the 5 nodal lymphoma patients with γδ T-cell phenotype was within 3 months, which was inferior to those of αβ T and TCR-silent types (P=0.003), and 3 of those with available clinical information were all found to be associated with autoimmune diseases. These data suggest that nodal EBV CTL is distinct from ENKTL.
在爱泼斯坦-巴尔病毒（EBV）阳性的细胞毒性T/NK细胞淋巴瘤中，关于原发性淋巴结表现患者（淋巴结EBV细胞毒性T细胞淋巴瘤[CTL]）的临床病理特征的报道较少。在此，我们比较了39例淋巴结EBV CTL患者与27例鼻型“鼻外”NK/T细胞淋巴瘤（ENKTL）患者的临床病理特征，尤其关注其T细胞受体（TCR）表型。组织学上，39例淋巴结EBV CTL病例中有22例（56%）具有中心母细胞样外观，这与ENKTL中该特征的较低发生率形成对比（15%，P=0.001）。相反，多形性外观在ENKTL中比在淋巴结EBV CTL中更常见（67%对23%，P=0.001）。基于TCR表达和/或TCRγ基因重排，39例淋巴结EBV CTL病例中有33例（85%）为T细胞谱系；具体而言，18例（46%）为TCRβ阳性（αβ T），5例（13%）为TCRγ和/或δ阳性（γδ T），10例（26%）为TCR沉默型，伴有克隆性TCRγ基因重排但无TCRβ、γ或δ表达。这些结果与ENKTL中T细胞谱系的较低发生率形成明显对比（7例，26%，P<0.001）。值得注意的是，5例具有γδ T细胞表型的淋巴结淋巴瘤患者的生存时间在3个月以内，低于αβ T和TCR沉默型患者（P=0.003），并且3例有可用临床信息的患者均被发现与自身免疫性疾病有关。这些数据表明淋巴结EBV CTL与ENKTL不同。
