title: A case of fatal idiopathic enteritis and multiple opportunistic infections associated with dendritic cell deficiencies.
abstract: We present a case of an adult patient with new-onset severe, idiopathic, protein-wasting enteropathy, in whom an extensive immunological workup was performed. We found a lack of dendritic cell (DC) subsets in the blood and bowel, as well as elevated circulating TGF-beta levels and decreased numbers of circulating FOXP3+ regulatory T cells with diminished CTLA4 expression. She failed to respond to glucocorticoids and infliximab, and instead developed a constellation of opportunistic infections, including CMV ileitis, Mucormycosis, and Pneumocystis carinii pneumonia, and ultimately passed away. While the cause of her lack of DCs is unknown, this data suggests a key role for these cells in both regulating mucosal immunity and promoting effective cell-mediated immunity against pathogens in humans.
authors.affiliation: Translational Research Program Benaroya Research Institute, Seattle, WA 98101-2795, USA. james.lord@vmmc.org
authors.forename: James D
authors.initials: JD
authors.lastname: Lord
authors.forename: Janice
authors.initials: J
authors.lastname: Chen
authors.forename: Richard A
authors.initials: RA
authors.lastname: Kozarek
mesh_terms: D003106:Colon / Q000276:immunology; D016207:Cytokines / Q000097:blood; D003713:Dendritic Cells / Q000276:immunology*; D004751:Enteritis / Q000150:complications / Q000276:immunology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007082:Ileum / Q000276:immunology; D007111:Immunity, Cellular; D018928:Immunity, Mucosal; D015212:Inflammatory Bowel Diseases / Q000276:immunology; D008875:Middle Aged; D009894:Opportunistic Infections / Q000150:complications / Q000276:immunology*; D050378:T-Lymphocytes, Regulatory / Q000276:immunology
chemical_list: D016207:Cytokines
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