Toll-like receptor stimulation induces higher TNF-alpha secretion in peripheral blood mononuclear cells from patients with hyper IgE syndrome.

• Authors: YEGANEH Mehdi; HENNEKE Philip; REZAEI Nima; EHL Stephan; THIEL Doerte; MATAMOROS Nuria; PIETROGRADA Cristina; ESPANOL Teresa; LITZMAN Jiří; FRANCO Jose L.; SANAL Ozden; KILIC Sara S.; BREBOROWICZ Anna; BLEBANI Alessandro; RENNER Ellen; ROTHENFUSSER Simon; RAWN Thomas R.; WOELLNER Cristina; GRIMBACHER Bodo
• Year of publication: 2008
• Type: Article in Periodical
• Magazine / Source: International Archives Allergy and Immunology
• MU Faculty or unit: Faculty of Medicine
• Field: Immunology
• Keywords: Hyper IgE syndrome; Toll-like receptor;TNF alpha
• Description: 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.