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We report the case of a 67-year-old man with NK/T cell lymphoma of the larynx accompanied by hemophagocytic syndrome (HPS) who reported throat paresthesia and was referred for evaluation of a laryngeal tumor extending from the left false vocal cord to the arytenoids. Two separate biopsies from the larynx yielded a histological diagnosis of NK/T-cell lymphoma, nasal and nasal type. Since he showed pancytopenia and remittent fever soon after admission, bone marrow was examined and showed severe hemophagocytosis, leading to a diagnosis of HPS. He died of circulatory and respiratory distresses due to HPS about one month after admission. HPS develops with symptoms of fever, cytopenia, and liver dysfunction, and is characterized by systemic proliferation of benign hemophagocytic histiocytosis in the bone marrow, lymph nodes, liver, and spleen. To the best of our knowledge, this is only the second case of NK/T-cell lymphoma of the larynx with hemophagocytic syndrome to be reported in the Japanese literature.

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