Extranodal Natural Killer T-Cell Lymphoma
A 53-year-old man presented to the emergency department with a 1-month history of relapsing fevers, dyspnea, and rapidly progressive skin lesions. Physical examination revealed painful, indurated nodules with surrounding purple discoloration on his back (Panel A), trunk, and limbs. The results of laboratory investigations included a white-cell count of 3000 per cubic millimeter (reference range, 3400 to 9100), a platelet count of 36,000 per cubic millimeter (reference range, 150,000 to 450,000), and a ferritin level of more than 40,000 ng per milliliter (reference range, 22 to 275). Bone marrow aspirates showed hemophagocytosis, and a biopsy specimen obtained from a skin lesion showed extranodal natural killer (NK) T-cell lymphoma. Positron-emission tomography (PET) revealed increased fluorodeoxyglucose avidity in the nasopharynx, mediastinum, abdomen, pelvis, testes, and skin. The patient’s Epstein–Barr viral load was 18,486 U per milliliter. Extranodal NK T-cell lymphoma is an uncommon neoplasm that is associated with the Epstein–Barr virus. The patient received six courses of systemic chemotherapy with gemcitabine, cisplatin, dexamethasone, and asparaginase. At follow-up at 8 months, there was a dramatic improvement in the condition of his skin (Panel B), and the Epstein–Barr virus was undetectable. Resolution of the fluorodeoxyglucose-avid lesions was observed on a restaging PET scan.
Lymphoma