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Extra-Nodal, Nasal, Natural Killer T-Cell Lymphoma Treated With a Checkpoint Inhibitor: A Case Report of a Sustained Complete Response

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 stag...

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Detalles Bibliográficos
Autores principales: Diab, Radwan, Kamran, Syed, Adcock, Bridget, Choucair, Khalil, Truong, Quoc V
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8141688/
https://www.ncbi.nlm.nih.gov/pubmed/34046285
http://dx.doi.org/10.7759/cureus.14654
Descripción
Sumario: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.