title: Aggressive mature natural killer cell neoplasms: report on a series of 12 European patients with emphasis on flow cytometry based immunophenotype and DNA content of neoplastic natural killer cells.
abstract: We report 12 cases of aggressive natural killer (NK) cell neoplasms diagnosed in Portugal, with emphasis on flow cytometry. Ten patients had extranodal NK/T cell lymphoma, nasal type and two had aggressive NK cell leukemia, and seven were men and five were women, with a median age of 50 years. NK cells brightly expressed the CD56 adhesion molecule and CD94 lectin type killer receptor and had an activation-related HLA-DR+ CD45RA+ CD45RO+ immunophenotype, in most cases. In contrast, dim CD16 expression was found in a minor proportion of cases, whereas CD57 and the CD158a and CD158e1 killer immunoglobulin-like receptors were negative. One-third of cases showed a hyperploid DNA content and nearly all had a very high S-phase proliferative rate. The phenotypic features of the neoplastic NK cells would suggest that they represent the transformed counterpart of the CD56 + bright NK cells that circulate in normal blood.
authors.affiliation: Laboratory of Cytometry, Department of Hematology.
authors.forename: Margarida
authors.initials: M
authors.lastname: Lima
authors.forename: Ana
authors.initials: A
authors.lastname: Spínola
authors.forename: Sónia
authors.initials: S
authors.lastname: Fonseca
authors.forename: Ana Helena
authors.initials: AH
authors.lastname: Santos
authors.forename: João
authors.initials: J
authors.lastname: Rodrigues
authors.forename: Lurdes
authors.initials: L
authors.lastname: Oliveira
authors.forename: Maria Luís
authors.initials: ML
authors.lastname: Queirós
authors.forename: Marlene
authors.initials: M
authors.lastname: Santos
authors.forename: Marta
authors.initials: M
authors.lastname: Gonçalves
authors.forename: Catarina
authors.initials: C
authors.lastname: Lau
authors.forename: Maria dos Anjos
authors.initials: Mdos A
authors.lastname: Teixeira
authors.forename: Cristina
authors.initials: C
authors.lastname: Gonçalves
authors.forename: Cristina
authors.initials: C
authors.lastname: Marques
authors.forename: Manuel
authors.initials: M
authors.lastname: Guerreiro
authors.forename: Manuel
authors.initials: M
authors.lastname: Cunha
authors.forename: Fernando
authors.initials: F
authors.lastname: Príncipe
authors.forename: Jorge
authors.initials: J
authors.lastname: Coutinho
mesh_terms: D000328:Adult; D000368:Aged; D000954:Antigens, Surface / Q000378:metabolism; D003131:Combined Modality Therapy; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000378:metabolism / Q000473:pathology; D054066:Leukemia, Large Granular Lymphocytic / Q000175:diagnosis* / Q000628:therapy; D054391:Lymphoma, Extranodal NK-T-Cell / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000954:Antigens, Surface
keywords: Aggressive NK cell leukemia; CD56 + bright NK cells; DNA content and cell cycle; NK cell immunophenotyping; NK/T cell lymphoma; flow cytometry; nasal type