Extranodal natural killer/T-cell lymphoma of the nasal cavity is a rare malignancy with poor overall prognosis. We report the case of a rapidly fatal natural killer/T-cell lymphoma of the nasal cavity complicated by the concominant development of hemophagocytic lymphohistiocytosis. This disorder is marked by clinical findings such as fever and splenomegaly, a multitude of abnormal laboratory findings, and a profound proliferation of circulating macrophages. Left untreated, multi-organ failure and death are common. Prompt diagnosis is essential to the successful management of this disorder and for subsequent recovery.
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