Li Lin-Jie, Zhang Jun-Yu
Department of Hematology, Lishui Municipal Central Hospital, Lishui 323000, Zhejiang Province, China.
World J Clin Cases. 2022 Oct 6;10(28):10193-10200. doi: 10.12998/wjcc.v10.i28.10193.
Extranodal natural killer/T cell lymphoma, nasal type (ENKL) is a highly aggressive malignancy characterized by its association with Epstein-Barr virus (EBV) and extranodal involvement, which shows a poor clinical outcome. Although L-asparaginase-based chemotherapy has improved the response rates of relapsed/refractory (R/R) ENKL, relapse occurs in up to 50% of patients with disseminated disease.
Immune evasion has emerged as a critical pathway for survival in ENKL and may be effectuated STAT3-driven upregulation of programmed cell death ligand 1 (PD-L1) or other molecular pathways. Anti-PD-1 is effective for R/R ENKL with EBV-driven upregulation of PD-L1 expression. Anti-PD-1 combined with decitabine showed positive preliminary results in a patient with R/R ENKL and resistance to anti-PD-1.
The treatment experience, in this case, demonstrated the potential ability of decitabine combined with PD-1 inhibitor to treat R/R ENKL, thus providing a new treatment strategy for this tumor.
结外鼻型自然杀伤/T细胞淋巴瘤（ENKL）是一种侵袭性很强的恶性肿瘤，其特征是与爱泼斯坦-巴尔病毒（EBV）相关且累及结外组织，临床预后较差。尽管基于左旋门冬酰胺酶的化疗提高了复发/难治性（R/R）ENKL的缓解率，但高达50%的播散性疾病患者会出现复发。
免疫逃逸已成为ENKL生存的关键途径，可能通过信号转导和转录激活因子3（STAT3）驱动的程序性细胞死亡配体1（PD-L1）上调或其他分子途径实现。抗PD-1对EBV驱动的PD-L1表达上调的R/R ENKL有效。抗PD-1联合地西他滨在一名R/R ENKL且对抗PD-1耐药的患者中显示出阳性初步结果。
该病例的治疗经验证明了地西他滨联合PD-1抑制剂治疗R/R ENKL的潜在能力，从而为该肿瘤提供了一种新的治疗策略。
