Diab Radwan, Kamran Syed, Adcock Bridget, Choucair Khalil, Truong Quoc V
Internal Medicine, University of Kansas School of Medicine, Wichita, USA.
Hematology/Oncology, Cancer Center of Kansas, Wichita, USA.
Cureus. 2021 Apr 23;13(4):e14654. doi: 10.7759/cureus.14654.
Extra-nodal natural killer T-cell lymphoma (ENKTL) is a rare and aggressive hematologic malignancy found in the nasal cavity and adjacent locations in 80% of cases, accounting for approximately 10% of non-Hodgkin lymphoma (NHL) and 0.4% of all cancers. Prognosis is typically poor and depends on stage, location, age, and tumor markers for targeted therapy, which is reserved for relapsed/refractory ENKTL. Of those, advanced clinical stage, higher Prognostic Index (PI), nodal involvement, Ki-67 expression, large cells, local tumor invasiveness, and circulating Epstein-Barr virus (EBV)-DNA levels are predictive of worse survival. Here, we present a rare case of a patient in remission 30 months after diagnosis of ENKTL following a sustained complete response to pembrolizumab, an immune checkpoint inhibitor targeting the programmed death-1 (PD-1) receptor.
结外自然杀伤T细胞淋巴瘤（ENKTL）是一种罕见且侵袭性强的血液系统恶性肿瘤，80%的病例发生于鼻腔及相邻部位，约占非霍奇金淋巴瘤（NHL）的10%，占所有癌症的0.4%。其预后通常较差，取决于分期、部位、年龄以及用于靶向治疗的肿瘤标志物，靶向治疗仅适用于复发/难治性ENKTL。其中，晚期临床分期、较高的预后指数（PI）、淋巴结受累、Ki-67表达、大细胞、局部肿瘤侵袭性以及循环 Epstein-Barr病毒（EBV）-DNA水平提示生存预后较差。在此，我们报告一例罕见病例，患者诊断为ENKTL后，对靶向程序性死亡-1（PD-1）受体的免疫检查点抑制剂派姆单抗持续完全缓解，30个月后病情缓解。
