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Extranodal natural killer/T-cell lymphoma (ENKL), nasal type, is a rare, aggressive non-Hodgkin lymphoma for which no clear standard of care has been established, particularly in the relapsed/refractory disease setting. Because of its rarity, randomized trials are not conducted specifically on ENKL, nasal type; however, case reports and small case series can provide important insights into potential new treatments. We present a case report of a patient with ENKL, nasal type (previously misdiagnosed as relapsing chronic sinusitis), whose disease progressed during multi-agent chemotherapy but responded to second-line treatment with single-agent pralatrexate. We discuss treatment options for relapsed/refractory ENKL, nasal type, and suggest that pralatrexate be further evaluated in this clinical setting.

Misdiagnosed Refractory Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type, Successfully Treated: A Case Report