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

作者：Yeganeh Mehdi; Henneke Philipp; Rezaei Nima; Ehl Stephan; Thiel Doerte; Matamoros Nuria; Pietrogrande Cristina; Espanol Teresa; Litzman Jiri; Franco Jose L; S**** Ozden; Kilic Sara S; Breborowicz Anna; Plebani Alessandro; Renner Ellen; Rothenfusser Simon; Hawn Thomas R; Woellner Cristina; Grimbacher Bodo^*

来源：International Archives of Allergy and Immunology, 2008, 146(3): 190-194.

DOI：10.1159/000115886

• hyper IgE syndrome
• toll-like receptor
• TNF-alpha

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.

• 出版日期2008