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

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.