Interleukin-18 in Hemophagocytic Lymphohistiocytosis

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Abstract
Hemophagocytic lymphohistiocytosis (HLH) is characterized by dysregulated hyperactivation of macrophages and T helper 1 (Th1) cells accompanied by excessive secretion of inflammatory cytokines. Although TNF-α and IFN-γ are known to be important factors for the development of the disease, the mechanism of their overproduction has not been clarified, yet. We measured serum IL-18 levels of patients with HLH to investigate the possible significance of IL-18 in its pathophysiology, especially in IFN-γ production. IL-18 levels were significantly increased in all patients with HLH compared with healthy controls. A significant correlation was observed between EL-18 and IFN-γ levels. In addition to IFN-γ and soluble Fas ligand (sFasL), IL-18 levels significantly correlated with disease activity. IL-18 may play important roles in the pathogenesis of HLH, particularly through induction of Th1 cells. In addition, IL-18 measurement may not only be useful for the diagnosis, but also for the evaluation of disease activity.

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