title: Primary CD56 + nasal-type T/natural killer-cell subcutaneous panniculitic lymphoma: presentation as haemophagocytic syndrome.
abstract: Natural killer (NK) cells are large granular lymphocytes that mediate cytotoxic reactions which are not restricted by the major histocompatibility complex. In recent years it has become apparent that a minor proportion of malignant lymphomas expresses an NK-cell phenotype defined by its reactivity with the CD56 antibody. Primary purely cutaneous CD56 + lymphomas have rarely been reported. They share a generally aggressive course and are highly associated with Epstein-Barr virus. We describe a patient with a primary cutaneous nasal-type T/NK-cell lymphoma that presented as a haemophagocytic syndrome and showed an aggressive clinical course.
authors.affiliation: Department of Dermatology, Asan Medical Centre, College of Medicine, University of Ulsan, Seoul, Korea.
authors.forename: K A
authors.initials: KA
authors.lastname: Jang
authors.forename: J H
authors.initials: JH
authors.lastname: Choi
authors.forename: K J
authors.initials: KJ
authors.lastname: Sung
authors.forename: K C
authors.initials: KC
authors.lastname: Moon
authors.forename: J K
authors.initials: JK
authors.lastname: Koh
authors.forename: Y M
authors.initials: YM
authors.lastname: Kwon
authors.forename: H S
authors.initials: HS
authors.lastname: Chi
mesh_terms: D000328:Adult; D019002:CD56 Antigen; D004279:DNA, Viral / Q000032:analysis; D017809:Fatal Outcome; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology* / Q000276:immunology / Q000821:virology; D006801:Humans; D017403:In Situ Hybridization; D007694:Killer Cells, Natural; D016410:Lymphoma, T-Cell, Cutaneous / Q000150:complications* / Q000276:immunology / Q000821:virology; D009669:Nose Neoplasms / Q000150:complications* / Q000276:immunology / Q000821:virology; D012878:Skin Neoplasms / Q000150:complications* / Q000276:immunology / Q000821:virology
chemical_list: D019002:CD56 Antigen; D004279:DNA, Viral