Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Thailand.
Diagn Pathol. 2011 Aug 25;6:79. doi: 10.1186/1746-1596-6-79.
Extranodal NK/T-cell lymphoma, nasal type (ENKTL) is not common worldwide, but it is the most common T- and NK-cell lymphomas in many Asian countries. Immunophenotypic profiles were studied based on limited series. The authors, therefore, studied on ENKTL according to characterize immunophenotypic profiles as well as the distribution of EBV subtype and LMP-1 gene deletion.
By using tissue microarray (TMA), immunohistochemical study and EBV encoded RNA (EBER) in situ hybridization were performed. T-cell receptor (TCR) gene rearrangement, EBV subtyping, and LMP-1 gene deletion were studied on the available cases.
There were 22 cases eligible for TMA. ENKTL were positive for CD3 (91%), CD5 (9%), CD7 (32%), CD4 (14%), CD56 (82%), TIA-1 (100%), granzyme B (95%), perforin (86%), CD45 (83%), CD30 (75%), Oct2 (25%), and IRF4/MUM1 (33%). None of them was positive for βF1, CD8, or CD57. TCR gene rearrangement was negative in all 18 tested cases. EBV was subtype A in all 15 tested cases, with 87% deleted LMP-1 gene. Cases lacking perforin expression demonstrated a significantly poorer survival outcome (p = 0.008).
The present study demonstrated TIA-1 and EBER as the two most sensitive markers. There were a few CD3 and/or CD56 negative cases noted. Interestingly, losses of CD45 and/or CD7 were not uncommon while Oct2 and IRF4/MUM1 could be positive in a subset of cases. Based on the present study in conjunction with the literature review, determination of PCR-based TCR gene rearrangement analysis might not be a useful technique for making diagnosis of ENKTL.
结外 NK/T 细胞淋巴瘤，鼻型（ENKTL）在世界范围内并不常见，但在许多亚洲国家却是最常见的 T 细胞和 NK 细胞淋巴瘤。免疫表型特征曾基于有限的病例系列进行过研究。因此，作者根据特征性免疫表型特征以及 EBV 亚型和 LMP-1 基因缺失的分布情况，对 ENKTL 进行了研究。
通过使用组织微阵列（TMA）、免疫组织化学研究和 EBV 编码 RNA（EBER）原位杂交进行研究。对现有病例进行了 T 细胞受体（TCR）基因重排、EBV 亚型和 LMP-1 基因缺失研究。
有 22 例病例符合 TMA 标准。ENKTL 阳性表达 CD3（91%）、CD5（9%）、CD7（32%）、CD4（14%）、CD56（82%）、TIA-1（100%）、颗粒酶 B（95%）、穿孔素（86%）、CD45（83%）、CD30（75%）、Oct2（25%）和 IRF4/MUM1（33%）。它们均不表达βF1、CD8 或 CD57。18 例检测的病例中 TCR 基因重排均为阴性。15 例检测的 EBV 均为亚型 A，87%缺失 LMP-1 基因。缺乏穿孔素表达的病例生存结局明显较差（p=0.008）。
本研究表明 TIA-1 和 EBER 是最敏感的两种标志物。有少数 CD3 和/或 CD56 阴性病例。有趣的是，CD45 和/或 CD7 的缺失并不少见，而 Oct2 和 IRF4/MUM1 在一部分病例中可能呈阳性。基于本研究并结合文献复习，基于 PCR 的 TCR 基因重排分析可能不是诊断 ENKTL 的有用技术。
