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Lymphoproliferative disease of 'lak cell' precursor large granular lymphocytes in association with celiac disease
Author(s) -
Lopez P.,
Morris D. G.,
Galbraith P. R.,
Lillicrap D. P.,
Pross H. F.
Publication year - 1993
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830430209
Subject(s) - immunology , lymphoproliferative disorders , medicine , cd16 , cd8 , lymphoma , natural killer cell , lymphocyte , immunophenotyping , cd3 , biology , cytotoxic t cell , immune system , antigen , in vitro , biochemistry
Abstract We have investigated a case of lymphoproliferative disease of large granular lymphocytes (LDGL) occurring in association with celiac disease, anemia, neutropenia, and carcinomas of the endometrium, breast, and skin. The large granular lymphocyte (LGL) proliferation was monoclonal, T cell in origin, with T cell receptor β‐chain gene rearrangement, and a CD3 + , CD8 + , CD16 ± phenotype. In spite of the high frequency of LGL, natural killer (NK) cell activity was absent. Stimulation with interleukin‐2 in vitro, however, resulted in high lymphokine‐activated killer (LAK) cell activity against NK‐resistant targets. The T‐cell nature of the LAK precursor cells is in contrast to the majority seen in normal peripheral blood. Therapeutic trials of cyclosporin A, low‐dose cyclophosphamide, and levamisole were unsuccessful in reducing transfusion requirements. This case is unique in the association of LDGL with celiac disease. It is also unique in that the patient had been followed for several years prior to the onset of the LDGL. The case extends the list of lymphoproliferative disorders documented to be associated with celiac disease and, conversely, adds to our knowledge of lymphoproliferative disorder of LGL and its 'dysimmune' manifestations.