Published online Oct 6, 2022. doi: 10.12998/wjcc.v10.i28.10193
Peer-review started: April 9, 2022
First decision: June 16, 2022
Revised: June 24, 2022
Accepted: August 24, 2022
Article in press: August 24, 2022
Published online: October 6, 2022
Processing time: 167 Days and 4.5 Hours
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 via 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.
Core Tip: Extranodal natural killer/T cell lymphoma nasal type is a highly aggressive malignancy characterized by its association with Epstein-Barr virus and extranodal involvement, which shows a poor clinical outcome. Now, we report a rare case of relapsed/refractory classic Hodgkin lymphoma with resistance to anti-PD-1 in which anti-PD-1 combined with decitabine showed positive preliminary results. Our findings support the potential benefit of anti-PD-1 combined with decitabine in this type of refractory T-cell lymphoma.