Impaired type-1 activity and increased NK cells in Gleich's syndrome.

Abstract

An altered production of cytokines has been described in Gleich's syndrome. Our aim was to study the cytokine production at the single-cell level in a patient with Gleich's syndrome and to determine whether it changed during a flare episode. Blood samples were obtained from a 30-year-old woman diagnosed with Gleich's syndrome before and during the subsequent flare of edema of trunk and arms and weight gain. The major lymphoid subsets (CD4+, CD8+, and CD19+) and natural killer (NK) cells were measured by flow cytometry. Cytokine-producing T cells (IL-2, IFN-gamma, and IL-4) were quantified in whole blood by intracellular staining with specific monoclonal antibodies and flow cytometry analysis after polyclonal stimulation with phorbol 12-myristate 13-acetate and ionomycin. Increased numbers of immature CD4+CD8+ T cells and NK cells were observed in peripheral blood during the asymptomatic period. The latter population significantly decreased during the flare. Type-1 cells were decreased in both asymptomatic and, more markedly, during the attack with respect to healthy subjects. The decreased type-1 response demonstrated in this patient might be the basis of the hypereosinophilia of Gleich's syndrome. Besides, the NK cells might play a role in the pathogenesis of these inflammatory episodes.