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Esti Fitria
Krisna Murti

Non Hodgkin Lymphoma (NHL), especially Extranodal NK/T cell Lymphoma (ENKTL) is a rare malignancy with an incidence rate of approximately 12% (168 out of 1,403 cases) of all NHL cases.Research by Aozasa et al., the frequency of ENKTL is estimated to be 10-fold higher in Asian populations when compared to Europeans. According to WHO 2022, extranodal NK/T cell lymphoma, nasal type is often found in the respiratory tract, nasopharynx, paranasal sinuses, and palate. This type of lymphoma is closely associated with Epstein-Barr Virus (EBV) infection. Over the past decade, immunotherapy is, once again, a promising approach to cancer treatment, due to the remarkable salutary effectiveness of Immune-Checkpoint Inhibitors (ICIs) targeting Programmed Cell Death-1 (PD-1) or Programmed Cell Death Ligand-1 (PD-L1). Programmed Death-Ligand 1 (PD-L1) is an immune checkpoint protein that manipulates the host immune response by regulating T cell function. Programmed Death-Ligand 1 is overexpressed in EBV+ lymphoma cells especially in ENKTL compared to EBV negative lymphomas and is associated with worse prognosis. In addition to determining prognosis, PD-L1 inhibitors are used as cancer therapy. Programmed Death-Ligand 1 expressed by ENKTL tumor cells as a target for PD-L1 inhibitortherapy (pembrolizumab). Programmed cell death ligand 1 (PD-L1) overexpression was associated with the clinicopathological factors of B symptoms, IPI score, and Ann Arbor Stage. Some studies examined the expression of PD-1 and PDL1 in tumor cells and found PD-L1+ tumor cells was an independent poor prognostic factor for OS.

Keywords: Extranodal NK/T cell lymphoma Epstein-Barr Virus Programmed Death Ligand 1 Staging Ann Arbor Prognosis