Ko Y H, Ree H J, Kim W S, Choi W H, Moon W S, Kim S W
Department of Diagnostic Pathology, Sungkyunkwan University School of Medicine, Samsung Medical Center, Seoul, Korea.
Cancer. 2000 Nov 15;89(10):2106-16. doi: 10.1002/1097-0142(20001115)89:10<2106::aid-cncr11>3.0.co;2-g.
This study aimed to define genotypic profile and to describe the clinicopathologic features of nasal-type natural killer (NK)/T-cell lymphoma of nasal and extranasal origin and NK precursor lymphoma.
NK/T-cell lymphomas from the upper aerodigestive tract (n = 45), skin (n = 2), gastrointestinal tract (n = 3), and soft tissue (n = 2) and NK precursor neoplasms (n = 3) were studied. Immunophenotype was analyzed by immunohistochemistry and flow cytometry. In situ hybridization with EBER 1/2 RNA probes was performed. T-Cell Receptor (TCR)-gamma gene rearrangement was analyzed by seminested polymerase chain reaction with heteroduplex analysis. Overall survival rate was correlated with clinicopathologic parameters and compared by Wilcoxon test.
Clonal TCR-gamma gene rearrangement was detected in 3 of 31 upper aerodigestive and 1 of 2 skin tumors. When immunostained using paraffin embedded tissue, 6 upper aerodigestive lymphomas were negative for CD56 in which 4 cases lacked clonal TCR gene rearrangement. Epstein-Barr virus (EBV) mRNA was detected in 33 upper aerodigestive tumors including 26 of 29 nasal tumors (90%), and 7 of 10 extranasal tumors (70%). There was no histologic, immunophenotypic, or genotypic differences according to the lineage and EBV association in upper aerodigestive lymphomas. Among the patients with upper aerodigestive tumors, overall 1-year survival rate was 41%, and correlated well with the stage (P < 0.05) but not with the size of tumor cells, EBV status, and lineage (P > 0.05). Median survival rate of lymphomas from other sites excluding upper aerodigestive tract was not significantly different from that of upper aerodigestive lymphomas with same stage (P > 0.05). Unlike nasal-type NK/T-cell lymphomas, NK precursor lymphoma involved the bone marrow and lymph nodes at initial presentation or in the course of disease. Tumor cells were positive for TdT in all and myeloid markers in two. TCR gene rearrangement was germ line.
Most upper aerodigestive nasal-type NK/T-cell lymphomas among Koreans are genotypically of NK derivation and few belong to T lineage. Presence or absence of EBV has no significant correlation with the histologic changes and the lineage of these lymphomas.
本研究旨在明确鼻型自然杀伤（NK）/T细胞淋巴瘤及鼻外来源的NK/T细胞淋巴瘤和NK前体淋巴瘤的基因型特征，并描述其临床病理特征。
研究了来自上呼吸消化道（n = 45）、皮肤（n = 2）、胃肠道（n = 3）和软组织（n = 2）的NK/T细胞淋巴瘤以及NK前体肿瘤（n = 3）。通过免疫组织化学和流式细胞术分析免疫表型。采用EBER 1/2 RNA探针进行原位杂交。通过半巢式聚合酶链反应和异源双链分析检测T细胞受体（TCR）-γ基因重排。总生存率与临床病理参数相关，并通过Wilcoxon检验进行比较。
在31例上呼吸消化道肿瘤中的3例以及2例皮肤肿瘤中的1例检测到克隆性TCR-γ基因重排。当使用石蜡包埋组织进行免疫染色时，6例上呼吸消化道淋巴瘤CD56呈阴性，其中4例缺乏克隆性TCR基因重排。在33例上呼吸消化道肿瘤中检测到爱泼斯坦-巴尔病毒（EBV）mRNA，包括29例鼻肿瘤中的26例（90%）和10例鼻外肿瘤中的7例（70%）。上呼吸消化道淋巴瘤在组织学、免疫表型或基因型上，根据谱系和EBV关联情况无差异。在上呼吸消化道肿瘤患者中，总体1年生存率为41%，与分期密切相关（P < 0.05），但与肿瘤细胞大小、EBV状态和谱系无关（P > 0.05）。除上呼吸消化道外其他部位淋巴瘤的中位生存率与相同分期的上呼吸消化道淋巴瘤无显著差异（P > 0.05）。与鼻型NK/T细胞淋巴瘤不同，NK前体淋巴瘤在初诊时或病程中累及骨髓和淋巴结。肿瘤细胞全部TdT呈阳性，2例髓系标志物呈阳性。TCR基因重排为种系。
韩国人中大多数上呼吸消化道鼻型NK/T细胞淋巴瘤在基因型上源自NK，少数属于T谱系。EBV的存在与否与这些淋巴瘤的组织学改变和谱系无显著相关性。
