Department of Anatomic Pathology, Chang Gung Memorial Hospital and Chang Gung University School of Medicine, 33305 Taoyuan, Taiwan.
Best Pract Res Clin Haematol. 2013 Mar;26(1):57-74. doi: 10.1016/j.beha.2013.04.006. Epub 2013 May 23.
Peripheral NK/T-cell lymphoma (PTCL) is a heterogeneous group of uncommon hematologic malignancies with aggressive clinical course and unfavorable prognosis. Extranodal NK/T-cell lymphoma, nasal type (NKTCL) is the most common extranodal entity worldwide, with heterogeneous geographic distribution, and it is characterized by its association with EBV, a nasal or less often extranasal presentation and aggressive behavior. Recent works using array-based technologies have provided novel insights into the pathogenesis and discovered new biomarkers with diagnostic and therapeutic implications in NKTCL. Gene expression profiling identified that most of the NKTCL are derived from activated natural killer cells with distinctively high expression of granzyme H compared to other PTCLs, which might serve as a new diagnostic biomarker. Frequent deletions and promoter methylations in PRDM1, ATG5, AIM1, FOXO3, HACE1 mapping to 6q21-q25, suggest their roles as potential tumor suppressors. The deregulation of oncogenic pathways (PDGF, JAK-STAT, AKT) provides a rationale for developing targeted therapies in the future.
外周 NK/T 细胞淋巴瘤（PTCL）是一组罕见的血液恶性肿瘤，具有侵袭性临床病程和不良预后。结外 NK/T 细胞淋巴瘤，鼻型（NKTCL）是全球最常见的结外实体，具有异质性的地理分布，其特征是与 EBV 相关，表现为鼻腔或较少见的鼻腔外表现和侵袭性行为。最近使用基于阵列的技术的研究为 NKTCL 的发病机制提供了新的见解，并发现了具有诊断和治疗意义的新生物标志物。基因表达谱分析表明，大多数 NKTCL 来源于活化的自然杀伤细胞，与其他 PTCL 相比，颗粒酶 H 的表达明显升高，这可能成为一种新的诊断生物标志物。PRDM1、ATG5、AIM1、FOXO3 和 HACE1 基因在 6q21-q25 上的频繁缺失和启动子甲基化提示它们可能作为潜在的肿瘤抑制因子发挥作用。致癌途径（PDGF、JAK-STAT、AKT）的失调为未来开发靶向治疗提供了依据。
