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 showi ng typical morphological features, expressing antigens characteristic for c ytotoxic T cells and exhibiting marked natural killer-like cytotoxicity tow ards different target cells. Moreover, characterization of the T-cell recep tors revealed simultaneous expression of two different types of beta -chain s 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 cli nical course hardly responsive to chemotherapeutic treatment. This is the f irst description of a peripheral T-cell neoplasm expressing four distinct t ypes of T-cell receptor molecules.