A Rare Case of Aggressive Disseminated Nasal-Type Epstein-Barr Virus-Positive Extranodal Natural Killer/T-Cell Lymphoma with Bone Marrow Involvement

Abstract

Patient: Female, 38-year-old Final Diagnosis: Nasal-type extranodal natural killer/T-cell lymphoma Symptoms: Facial pain • dyspnea • non-productive cough • nausea • vomiting • green nasal discharge • dark-brown eye discharge Clinical Procedure: — Specialty: Oncology Objective: Rare disease Background: Nasal-type extranodal natural killer/T-cell lymphoma (ENKL) is an exceedingly rare and aggressive subtype of non-Hodgkin lymphoma. The malignancy has both a high morbidity and mortality and is most commonly discovered in patients with advanced stages of the disease. As a result, early detection and treatment is tantamount to improving survival and minimizing lasting effects. Case Report: Herein, we report a case of nasal-type ENKL in a woman with facial pain and associated nasal and eye discharge. We highlight the histopathologic features from nasopharyngeal and bone marrow biopsy, which demonstrated Epstein-Barr virus-positive biomarkers of diffuse and subtle involvement, respectively, with associated chromogenic immunohistochemical staining. We also highlight existing therapy utilizing a combination of chemotherapy with radiation, as well as consolidation therapy, and suggest the need for further research of allogeneic hematopoietic stem cell treatment and the potential of programmed death ligand 1 (PD-L1) inhibition in managing nasal-type ENKL malignancy. Conclusions: Nasal-type ENKL is a rare subtype of non-Hodgkin lymphoma that is infrequently associated with bone marrow involvement. The malignancy has a poor prognosis overall and typically is discovered late in the disease course. Current treatment favors utilization of combined modality therapy. However, previous studies have been inconsistent in determining whether chemotherapy or radiation therapy can be used alone. Additionally, promising results have also been shown with chemokine modulators, including antagonistic drugs that target PD-L1, in refractory and advanced cases.