title: Aggressive NK cell lymphoma preceded by a ten year history of neutropenia associated with large granular lymphocyte lymphocytosis.
abstract: We report a case of aggressive natural killer (NK) cell lymphoma in an 82 year old man who first presented 10 years earlier with neutropenia in association with a large granular lymphocyte (LGL) lymphocytosis. The diagnosis of NK cell lymphoma was made on the basis of morphological and immunological characteristics (CD3-CD56+) found on skin biopsy of one of multiple skin nodules which subsequently developed in association with splenomegaly, thrombocytopenia and continuing neutropenia. In addition there was BM infiltration and a cytogenetic abnormality [add(6)(p25)] was detected. Combination chemotherapy led to an initial clinical response but a relapse occurred shortly afterwards and the patient died 8 months later from infection whilst neutropenic following re-introduction of chemotherapy. Previously reported cases of aggressive NK cell lymphoma have shown a young male predominance with a rapidly progressive clinical course and without evidence of a preceding chronic phase of LGL lymphocytosis and neutropenia.
authors.affiliation: Department of Haematology, St Helier Hospital, Surrey, UK.
authors.forename: C
authors.initials: C
authors.lastname: De Lord
authors.forename: J
authors.initials: J
authors.lastname: Mercieca
authors.forename: M
authors.initials: M
authors.lastname: Ashton-Key
authors.forename: L
authors.initials: L
authors.lastname: Singh
authors.forename: S
authors.initials: S
authors.lastname: Ryley
authors.forename: P
authors.initials: P
authors.lastname: Isaacson
authors.forename: D
authors.initials: D
authors.lastname: Catovsky
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D002869:Chromosome Aberrations; D002896:Chromosomes, Human, Pair 6; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D008218:Lymphocytosis / Q000150:complications*; D016399:Lymphoma, T-Cell / Q000209:etiology* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D008297:Male; D009503:Neutropenia / Q000150:complications*; D012878:Skin Neoplasms / Q000209:etiology* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology