title: Aggressive natural killer cell leukaemia/lymphoma: report of four cases and review of the literature. Possible existence of a new clinical entity originating from the third lineage of lymphoid cells.
abstract: The morphologic, immunologic, genotypic and functional properties of peripheral blood and bone marrow cells or cultured cells from four patients with a clinically aggressive non-T, non-B natural killer cell leukaemia/lymphoma (ANKL/L) are described. The leukaemic cells possessed medium to large granules in the cytoplasm, antigens against CD38, CD2, OKIa 1 and NKH-1 CD56) monoclonal antibodies on their cell-surface, and also showed natural killer (NK) activity. In addition, these ANKL/L belonged to neither T- nor B-cell lineage, proved by studying clonal gene rearrangement for the T beta, T gamma and T delta receptors, and immunoglobulin. After comparing them with the seven cases of ANKL/L reported in other institutions, with regard to immunophenotype, genotype and function, we conclude that ANKL/L originating from a third lineage of lymphoid cells is a distinct clinical entity.
authors.affiliation: Department of Internal Medicine, Hiroshima University, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Imamura
authors.forename: Y
authors.initials: Y
authors.lastname: Kusunoki
authors.forename: K
authors.initials: K
authors.lastname: Kawa-Ha
authors.forename: K
authors.initials: K
authors.lastname: Yumura
authors.forename: J
authors.initials: J
authors.lastname: Hara
authors.forename: K
authors.initials: K
authors.lastname: Oda
authors.forename: K
authors.initials: K
authors.lastname: Abe
authors.forename: H
authors.initials: H
authors.lastname: Dohy
authors.forename: T
authors.initials: T
authors.lastname: Inada
authors.forename: H
authors.initials: H
authors.lastname: Kajihara
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte / Q000235:genetics; D005838:Genotype; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D007938:Leukemia / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008223:Lymphoma / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D010641:Phenotype