혈액식작용 증후군을 동반한 구인두의 T/NK세포 림프종

T/NK-cell lymphoma (TNKL) is a subtype of angiocentric T-cell lymphoma showing the characteristics of biphenotype of T-cell and NK-cell, frequent association with Epstein-Barr virus (EBV), and poor clinical course. It usually develops in nasal cavity, paranasal sinus, and nasopharynx. Hemophagocytotic syndrome is a histiocytic proliferation associated with phagocytosis of the hemopoietic elements resulting in fever, profound pancytopenia, hepatosplenomegaly, lymphadenopathy, and coagulopathy. It is considered as a negative prognostic factor for hematologic neoplasms. Epstein-Barr virus infection is considered as pathogenic factor for hemophagocytotic syndrome. We experienced a case of EBV-infected oropharyngeal lymphoma of T/NK-cell lymphoma associated with hemophagocytotic syndrome, which was confirmed by immunohistochemical study, in situ hybridization, and bone marrow biopsy. We report this case with brief review of literatures. (J Clinical Otolaryngol 2001;12:83-88)