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
Citation
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.