P. Lopez et al., LYMPHOPROLIFERATIVE DISEASE OF LAK CELL PRECURSOR LARGE GRANULAR LYMPHOCYTES IN ASSOCIATION WITH CELIAC-DISEASE, American journal of hematology, 43(2), 1993, pp. 116-122
We have investigated a case of lymphoproliferative disease of large gr
anular 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 beta-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-activate
d 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-d
ose cyclophosphamide, and levamisole were unsuccessful in reducing tra
nsfusion requirements. This case is unique in the association of LDGL
with celiac disease. It is also unique in that the patient had been fo
llowed for several years prior to the onset of the LDGL. The case exte
nds the list of lymphoproliferative disorders documented to be associa
ted with celiac disease and, conversely, adds to our knowledge of lymp
hoproliferative disorder of LGL and its ''dysimmune'' manifestations.