T-large granular lymphocyte leukaemia with natural killer cell-like cytotoxicity and expression of two different alpha- and beta-T-cell receptor chains.
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.
Boehrer
S
S
Department of Medicine III, Johann Wolfgang Goethe-University Hospital, Frankfurt am Main, Germany.
Hinz
T
T
Schui
D
D
Harder
S
S
Chow
K U
KU
Schneider
B
B
Hoelzer
D
D
Mitrou
P S
PS
Weidmann
E
E
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
D016693:Receptors, Antigen, T-Cell, alpha-beta