Behcet's-like syndrome associated with idiopathic CD4(+) T-lymphocytopenia, opportunistic infections, and a large population of TCR alpha beta(+) CD4(-) CD8(-) T cells

Herein we report a patient with Behcet's like syndrome, idiopathic CD4(+) T-lymphocytopenia, opportunistic infections, and a large polyclonal population of TCR alpha beta(+) CD4(-) CD8(-) T cells, Microfluorimetric analysis of peripheral blood mononuclear cells revealed CD4(+) T-cell counts of 10 +/- 5/mm(3). The CD3(+) T cells were 99% TCR alpha beta(+), of which 74 +/- 5% were CD4(-) CD8(-). No clonal populations were detected by southern analysis for T-cell receptor V beta gene rearrangements, No evidence of human immunodeficiency virus infection was present, although nocardia, candida, pneumocystis, cytomegalovirus, and herpes infections were documented, The concomitant presence of opportunistic infections and a large population of TCR alpha beta(+) CD4(-) CD8(-) T cells suggests a pathogenic association and an intense immune response to microbial lipid or lipoglycan antigens presented in the context of CD1 molecules, This case demonstrates the potential for idiopathic CD4(+) T-lymphocytopenia to occur in Behcet's-like syndrome with lethal consequences.