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

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.