PROLIFERATION OF LARGE GRANULAR LYMPHOCYT ES IN PATIENTS WITH SYSTEMIC LUPUS-ERYTHEMATOSUS

Background. Large granular T lymphocytes (LGL) make up a small portion of cellular population in peripheral blood. An abnormal proliferation of LGL is detected together with cytopenic and other autoimmune disor ders and is often associated with rheumatoid arthritis. The associatio n with other autoimmune diseases, such as systemic lupus erythematosus , is poorly known. The clinical and immunological profile in five pati ents with SLE and LGL proliferation is here reported. Materials and me thods. A clinical follow-up and prospective phenotypic study of mononu clear cells was conducted in patients with SLE for a period of 24 mont hs. LGL were identified on the basis of their shape and analyzed by fl ow cytometry as cells coexpressing thymic differentiation antigens (CD 3 and CD4, or CD8) and NK cells CD16, CD56 or CD57). Results. Five out of 43 patients with SLE showed recurrent proliferations of LGL (from 2 to 4 per patient) chronologically associated with lupic exacerbation s. LGL represented 52 to 78% ((x) over bar+/-SD=56+/-8%) from the tota l of lymphocytes. The phenotype in proliferations was heterogeneous bu t it was consistent in later relapses in each patient (patient #1 and #2: CD3+CD8-CD4+CD16+CD56+CD57-HLA/DR+; patients #3 and #4: CD3+CD8+CD 4-CD16+CD56+CD57-HLA/DR+ patient #5: CD3+CD8+CD4-CD16+CD56+CD57-HLA/DR + patient 5:CD3+CD8+CD4-CD16+/-CD56-CD57+HLA/D R+). These fieve patien ts had long term SLE with a greater number of exacerbations and a tend ency to develop hemocytopenias, requiring high doses of corticosteroid s and even immunosuppressors to control their condition. Conclusions. Some patients with SLE develop LGL proliferations. The activity, clini cal severy and hematological involvement seem to be associated with th is immunological disorder, but the pathogenic significance and prognos is of these proliferations are still to be elucidated.