title: Aggressive sinonasal natural killer/T-cell lymphoma with hemophagocytic lymphohistiocytosis.
abstract: 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.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA. justin.turner@jhmi.edu
authors.forename: Justin H
authors.initials: JH
authors.lastname: Turner
authors.forename: Myriam
authors.initials: M
authors.lastname: Loyo
authors.forename: Sandra Y
authors.initials: SY
authors.lastname: Lin
mesh_terms: D003937:Diagnosis, Differential; D004724:Endoscopy; D017809:Fatal Outcome; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000473:pathology* / Q000628:therapy; D016399:Lymphoma, T-Cell / Q000473:pathology* / Q000628:therapy; D008297:Male; D055611:Natural Killer T-Cells / Q000473:pathology*; D009669:Nose Neoplasms / Q000473:pathology* / Q000628:therapy; D055815:Young Adult