Extranodal Natural Killer T-Cell Lymphoma

This article is an answer to the Case – Painful, Indurated Nodules with Purple Discoloration

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.