Department of Hematology, Fourth Hospital of Hebei Medical University, Shijiazhuang, China.
Neoplasma. 2020 May;67(3):636-643. doi: 10.4149/neo_2020_190307N194. Epub 2020 Feb 17.
Extranodal NK/T-cell lymphoma nasal type (ENKTL) is a subtype of T cell lymphoma with poor prognosis. In this study, we designed a new prognostic model specifically for ENKTL to improve the risk stratification. In 29 ENKTL patients, we screened mutations in 9 ENKTL-associated genes using next-generation sequencing (NGS). We have found that mutated KMT2D was associated with the inferior overall survival (OS) and progression free survival (PFS) and KMT2D or TP53 mutations were associated with a higher mortality rate. Moreover, the difference in PFS among different stratifications was not significant using the International Prognostic Index (IPI) alone but was significant after the mutation status of KMT2D and TP53 were incorporated into the IPI model, forming a harmonious risk stratification reflecting the clinical features and genetic information of ENKTL. In summary, we demonstrate that the prognostic value of the IPI system can be enhanced by integrating the status of genetic mutations.
结外 NK/T 细胞淋巴瘤鼻型（ENKTL）是 T 细胞淋巴瘤的一种亚型，预后较差。在这项研究中，我们设计了一种新的预后模型，专门用于 ENKTL，以改善风险分层。在 29 名 ENKTL 患者中，我们使用下一代测序（NGS）筛选了 9 种与 ENKTL 相关的基因中的突变。我们发现，突变型 KMT2D 与较差的总生存期（OS）和无进展生存期（PFS）相关，而 KMT2D 或 TP53 突变与更高的死亡率相关。此外，仅使用国际预后指数（IPI）进行分层时，PFS 之间的差异不显著，但在将 KMT2D 和 TP53 的突变状态纳入 IPI 模型后，差异变得显著，形成了一种反映 ENKTL 临床特征和遗传信息的和谐风险分层。总之，我们证明通过整合基因突变状态，IPI 系统的预后价值可以得到增强。
