Article révisé par les pairs
Résumé : Interleukin-5 produced by Th2 lymphocytes is involved in the pathogenesis of a number of hypereosinophilic disorders. We and others have identified phenotypically abnormal clonal Th2 cells in peripheral blood of certain patients presenting the idiopathic hypereosinophilic syndrome (IHS). The premalignant nature of the aberrant lymphocytes is suggested by the occasional development of a peripheral T cell lymphoma bearing the same phenotype. In our series of five patients, the T cell clones all bore a CD3- CD4+ phenotype. The production of type-2 cytokines and the proliferation of these cells in response to dendritic cells in vitro were dependent on engagement of CD2 and CD28 molecules and on an IL-2/IL-2R autocrine loop. The high-level spontaneous apoptosis displayed by these cells in vitro was drastically inhibited by IL-2 and IFN-α, suggesting that administration of IFN-α to such patients could favour further expansion and eventual malignant transformation of the T cell clone. The hypereosinophilic syndrome may represent an unexpected application of new immunomodulatory molecules such as CTLA4-Ig and anti-IL-2R-α. © 2001 Éditions scientifiques et médicales Elsevier SAS.