Background: Pulmonary non-Hodgkin lymphoma (NHL) is rare. The most frequent subtype of pulmonary NHL is\nlow-grade B-cell lymphoma, such as lymphoma of mucosa-associated lymphoma tissue. Extranodal natural killer\ncell/T-cell lymphoma, nasal type (ENKL) is characterized by predominant extranodal involvement and association\nwith Epstein-Barr virus (EBV). ENKL with massive lung involvement has been infrequently reported, and its prognosis\nis extremely poor.\nCase presentation: A 20-year-old Japanese man presented with intermittent fever lasting for 2 months. Radiological\nimaging demonstrated multiple nodules of uneven shape and size in both lungs. Video-assisted thoracic surgical lung\nbiopsy showed abnormal lymphocyte infiltration, which was positive for CD3, CD56, and perforin. In situ hybridization\nfor EBV-encoded RNA was positive. From these findings, he was diagnosed with ENKL with lung involvement. The\npatient was successfully treated with intensive combinational chemotherapy followed by allogeneic cord blood\ntransplantation. He has been alive with continuous complete remission for 1 year after diagnosis.\nConclusions: Although ENKL involving the lung has been reported to have dismal outcomes, our patient showed\nlong-term survival after intensive chemotherapy and up-front allogeneic hematopoietic transplantation. The present\ncase highlights the importance of early diagnosis as well as allogeneic transplantation.
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