title: Aggressive natural killer cell leukaemia: a rare and fatal disorder.
abstract: Natural killer (NK) cell neoplasms, which include extra-nodal NK/T-cell lymphoma (nasal and extra-nasal) and aggressive NK cell leukaemia, are generally rare, but they are more common in people of Oriental, Mexican and South American descent. These neoplasms are highly aggressive, and show a strong association with Epstein-Barr virus. Aggressive NK cell leukaemia affects younger patients, who present with poor general condition, fever, and disseminated disease; they often die within a short time from systemic disease or complications such as multi-organ failure. Aggressive NK cell leukaemia must be distinguished from T-cell large granular lymphocyte leukaemia and indolent NK cell lympho-proliferative disorder, both of which are indolent. We present a case of young Asian male with aggressive NK cell leukaemia who presented with a poor general condition and disseminated disease. The patient had a rapidly progressive disease and died within weeks of diagnosis.
authors.affiliation: Department of Medicine, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi-110 060.
authors.forename: A
authors.initials: A
authors.lastname: Gogia
authors.forename: A
authors.initials: A
authors.lastname: Kakar
authors.forename: S P
authors.initials: SP
authors.lastname: Byotra
authors.forename: M
authors.initials: M
authors.lastname: Bhargav
mesh_terms: D000328:Adult; D000888:Antibodies, Anti-Idiotypic; D001706:Biopsy; D004358:Drug Therapy; D017809:Fatal Outcome; D005434:Flow Cytometry; D004854:Herpesvirus 4, Human / Q000276:immunology; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D007938:Leukemia / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D008223:Lymphoma / Q000473:pathology* / Q000628:therapy; D008297:Male
chemical_list: D000888:Antibodies, Anti-Idiotypic; C048844:anti-IgG