title: T-large granular lymphocyte leukaemia with natural killer cell-like cytotoxicity and expression of two different alpha- and beta-T-cell receptor chains.
abstract: We describe a case of cytotoxic T-large granular lymphocyte leukaemia showing typical morphological features, expressing antigens characteristic for cytotoxic T cells and exhibiting marked natural killer-like cytotoxicity towards different target cells. Moreover, characterization of the T-cell receptors revealed simultaneous expression of two different types of beta-chains as well as alpha-chains by the malignant cell clone. The patient had an 8 year history of indolent disease, before progressing to an aggressive clinical course hardly responsive to chemotherapeutic treatment. This is the first description of a peripheral T-cell neoplasm expressing four distinct types of T-cell receptor molecules.
authors.affiliation: Department of Medicine III, Johann Wolfgang Goethe-University Hospital, Frankfurt am Main, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Boehrer
authors.forename: T
authors.initials: T
authors.lastname: Hinz
authors.forename: D
authors.initials: D
authors.lastname: Schui
authors.forename: S
authors.initials: S
authors.lastname: Harder
authors.forename: K U
authors.initials: KU
authors.lastname: Chow
authors.forename: B
authors.initials: B
authors.lastname: Schneider
authors.forename: D
authors.initials: D
authors.lastname: Hoelzer
authors.forename: P S
authors.initials: PS
authors.lastname: Mitrou
authors.forename: E
authors.initials: E
authors.lastname: Weidmann
mesh_terms: D018450:Disease Progression; D005434:Flow Cytometry; D015332:Gene Rearrangement, alpha-Chain T-Cell Antigen Receptor; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology*; D015458:Leukemia, T-Cell / Q000235:genetics* / Q000276:immunology*; D017254:Leukemic Infiltration; D008297:Male; D008875:Middle Aged; D016693:Receptors, Antigen, T-Cell, alpha-beta; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013997:Time Factors
chemical_list: D016693:Receptors, Antigen, T-Cell, alpha-beta