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Yeganeh, Mehdi; Henneke, Philipp; Rezaei, Nima; Ehl, Stephan; Thiel, Doerte; Matamoros, Nuria; Pietrogrande, Cristina; Espanol, Teresa; Litzman, Jiri; Franco, Jose L.; Sanal, Ozden; Kilic, Sara S.; Breborowicz, Anna; Plebani, Alessandro; Renner, Ellen; Rothenfusser, Simon; Hawn, Thomas R.; Woellner, Cristina; Grimbacher, Bodo (2008): Toll-like receptor stimulation induces higher TNF-alpha secretion in peripheral blood mononuclear cells from patients with hyper IgE syndrome. In: International Archives of Allergy and Immunology, No. 3: pp. 190-194


Hyper IgE syndromes (HIES) are primary immunodeficiency disorders of unknown pathogenesis. Patients are typically affected with `cold' abscesses of the skin, recurrent cyst-forming pneumonia, chronic mucocutaneous candidiasis and other less frequent features such as progressive skeletal abnormalities. Defective signaling in the Toll-like receptor (TLR) pathways has been suggested as a responsible pathologic mechanism, however, in previous reports, 10 patients revealed no defect in inflammatory cytokine responses to different TLR ligands. Here, we report the increase in pro-inflammatory cytokines TNF-alpha and IL-8, following TLR2 and TLR4 stimulation in a larger cohort of 25 additional patients with HIES, and provide a meta-analysis of the TLR data in HIES. Copyright (C) 2008 S. Karger AG, Basel.