Abstract
BackgroundPulmonary non-Hodgkin lymphoma (NHL) is rare. The most frequent subtype of pulmonary NHL is low-grade B-cell lymphoma, such as lymphoma of mucosa-associated lymphoma tissue. Extranodal natural killer cell/T-cell lymphoma, nasal type (ENKL) is characterized by predominant extranodal involvement and association with Epstein-Barr virus (EBV). ENKL with massive lung involvement has been infrequently reported, and its prognosis is extremely poor.Case presentationA 20-year-old Japanese man presented with intermittent fever lasting for 2 months. Radiological imaging demonstrated multiple nodules of uneven shape and size in both lungs. Video-assisted thoracic surgical lung biopsy showed abnormal lymphocyte infiltration, which was positive for CD3, CD56, and perforin. In situ hybridization for EBV-encoded RNA was positive. From these findings, he was diagnosed with ENKL with lung involvement. The patient was successfully treated with intensive combinational chemotherapy followed by allogeneic cord blood transplantation. He has been alive with continuous complete remission for 1 year after diagnosis.ConclusionsAlthough ENKL involving the lung has been reported to have dismal outcomes, our patient showed long-term survival after intensive chemotherapy and up-front allogeneic hematopoietic transplantation. The present case highlights the importance of early diagnosis as well as allogeneic transplantation.
Highlights
WBC white blood cells, Neut neutrophils, Lymph lymphocytes, Mono monocytes, Abnormal lymph Abnormal lymphocytes, RBC Red blood cells, Hb hemoglobin, Plt platelet, PT prothrombin time, APTT activated partial thromboplastin time, Fib fibrinogen, TP total protein, Alb albumin, AST asparate aminotranferase, ALT alanine aminotransferase, LDH lactate dehydrogenase, T-bil total bilirubin, BUN blood urea nitrogen, Cre creatinine, CRP C-reactive protein, sIL-2R soluble IL-2 receptor, Ab antibody, Ag antigen, HB hepatitis B virus, HCV hepatitis C virus, HIV human immunodeficiency virus, EBV Epstein-Barr virus VCA; viral capsid antigen, EBNA EBV nuclear antigen cutaneous chronic Graft-versus-host disease (GVHD) limited to the head and neck, which could be managed with topical steroids
Discussions and conclusions ENKL is a rare type of lymphoma associated with EBV infection that most commonly involves the upper aerodigestive mucosa and skin
Localized ENKL has a better prognosis when treated with concurrent chemoradiotherapy [8], advanced ENKL often displays a fulminant clinical course with very poor prognosis
Summary
Conclusions: ENKL involving the lung has been reported to have dismal outcomes, our patient showed long-term survival after intensive chemotherapy and up-front allogeneic hematopoietic transplantation. We report a young Japanese man with pulmonary ENKL who was successfully treated with intensive combination chemotherapy and cord blood transplantation (CBT).
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