Abnormal proliferation of CD4(-) CD8(+) gamma delta(+) T cells with chromosome 6 anomaly: Role of fas ligand expression in spontaneous regression of the cells

We report a case of granular lymphocyte proliferative disorder accompanied with hemolytic anemia and neutropenia. Phenotypes of the cells were T cell receptor gamma delta(+) CD3(+) CD4(-)CD8(+) CD16(+) CD56(-) CD57(-), Southe rn blot analysis of T cell receptor beta and gamma chains demonstrated rear ranged bands in both. Chromosomal analysis after IL-2 stimulation showed de letion of chromosome 6, Sorted gamma delta(+) T cells showed an increase in Pas ligand expression compared with the levels in sorted alpha beta(+) T c ells, The expression of Pas ligand on these gamma delta(+) T cells increase d after IL-2 stimulation, The patient's anemia improved along with a decrea se in granular lymphocyte count and disappearance of the abnormal karyotype without treatment. The expression of Pas ligand may be involved in spontan eous regression of granular lymphocyte proliferation with hemolytic anemia, (C) 1999 Wiley-Liss, Inc.