title: Aggressive natural killer cell leukaemia/lymphoma in two patients with lethal midline granuloma.
abstract: We report two patients with leukaemic proliferations of large granular lymphocytes. The immunophenotype study showed that the leukaemic cells were positive for CD2, CD38, CD56 and anti-HLA-DR monoclonal antibodies and negative for other T-cell (CD3, CD4, CD8) and B-cell markers (CD19, CD20 and surface immunoglobulins). The clinical course was acute and a diagnosis of aggressive natural killer cell leukaemia/lymphoma was made. No clonal rearrangements of either C beta T-cell receptor or JH immunoglobulin genes were found. Functional studies done in one patient demonstrated non-restricted cytotoxic activity after activation with IL-2. Lethal midline granuloma had been previously diagnosed in both patients. A possible relationship between this entity and the natural killer cell leukaemia is discussed.
authors.affiliation: Servei d'Hematologia, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
authors.forename: J
authors.initials: J
authors.lastname: Soler
authors.forename: R
authors.initials: R
authors.lastname: Bordes
authors.forename: F
authors.initials: F
authors.lastname: Ortũno
authors.forename: M
authors.initials: M
authors.lastname: Montagud
authors.forename: J
authors.initials: J
authors.lastname: Martorell
authors.forename: C
authors.initials: C
authors.lastname: Pons
authors.forename: J
authors.initials: J
authors.lastname: Nomdedeu
authors.forename: J J
authors.initials: JJ
authors.lastname: Lopez-Lopez
authors.forename: J
authors.initials: J
authors.lastname: Prat
authors.forename: M
authors.initials: M
authors.lastname: Rutllant
mesh_terms: D015703:Antigens, CD / Q000032:analysis; D000951:Antigens, Neoplasm / Q000032:analysis; D005260:Female; D006103:Granuloma, Lethal Midline / Q000150:complications*; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D007945:Leukemia, Lymphoid / Q000209:etiology* / Q000276:immunology; D008297:Male; D008875:Middle Aged
chemical_list: D015703:Antigens, CD; D000951:Antigens, Neoplasm