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

Background: An altered production of cytokines has been described in Gleich 's syndrome. Our aim was to study the cytokine production at the single-cel l level in a patient with Gleich's syndrome and to determine whether it cha nged during a flare episode. Methods: Blood samples were obtained from a 30-year-old woman diagnosed wit h Gleich's syndrome before and during the Subsequent flare of edema of trun k and arms and weight gain. The major lymphoid subsets (CD4 +, CD8 +, and C D19 +) and natural killer (NK) cells were measured by flow cytometry. Cytok ine-producing T cells (IL-2, IFN-gamma, and IL-4) were quantified in whole blood by intracellular staining with specific monoclonal antibodies and flo w cytometry analysis after polyclonal stimulation with phorbol 12-myristate 13-acetate and ionomycin. Results: Increased numbers of immature CD4 + CD8 + T cells and NK cells wer e observed in peripheral blood during the asymptomatic period. The latter p opulation significantly decreased during the flare. Type-1 cells were decre ased in both asymptomatic arid, more markedly, during the attack with respe ct to healthy subjects. Conclusions: The decreased type-1 response demonstrated in this patient mig ht be the basis of the hypereosinophilia of Gleich's syndrome. Besides, the NK cells might play a role in the pathogenesis of these inflammatory episo des.