Primary CD56 + nasal-type T/natural killer-cell subcutaneous panniculitic lymphoma: presentation as haemophagocytic syndrome |
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Authors: | Jang K A Choi J H Sung K J Moon K C Koh J K Kwon Y M Chi H S |
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Institution: | Department of Dermatology, Asan Medical Centre, College of Medicine, University of Ulsan, Seoul, Korea. |
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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. |
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Keywords: | Haemophagocytic syndrome Primary cutaneous nasal-type T/NK-cell lymphoma |
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