title: Altered dendritic cell distribution in patients with common variable immunodeficiency.
abstract: Recent data suggest a critical role for dendritic cells (DCs) in the generation of immunoglobulin-secreting plasma cells. In the work reported herein, we analyzed the frequency of peripheral blood plasmacytoid DCs (pDCs) and myeloid DCs (mDCs) in a cohort of 44 adults with common variable immunodeficiency (CVID) classified according to their CD27 membrane expression status on B cells. A deep alteration in the distribution of DC subsets, especially of pDCs, in the peripheral blood of CVID patients was found. Patients with a reduced number of class-switched CD27+IgD-IgM- memory B cells and patients with granulomatous disease had a dramatic decrease in pDCs (P = 0.00005 and 0.0003 vs controls, respectively) and, to a lesser extent, of mDCs (P = 0.001 and 0.01 vs controls, respectively). In contrast, patients with normal numbers of switched memory B cells had a DC distribution pattern similar to that in controls. Taken together, our results raise the possibility that innate immunity contributes to pathogenesis in CVID.
authors.affiliation: Department of Internal Medicine and Infectious Diseases, Haut-Lévêque Hospital, Pessac, France. jean-francois.viallard@chu-bordeaux.fr
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Viallard
authors.forename: Fabrice
authors.initials: F
authors.lastname: Camou
authors.forename: Marc
authors.initials: M
authors.lastname: André
authors.forename: François
authors.initials: F
authors.lastname: Liferman
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Moreau
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Pellegrin
authors.forename: Patrick
authors.initials: P
authors.lastname: Blanco
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology / Q000473:pathology; D016175:B-Lymphocyte Subsets / Q000276:immunology; D001773:Blood Cells; D015331:Cohort Studies; D017074:Common Variable Immunodeficiency / Q000097:blood / Q000150:complications / Q000276:immunology* / Q000473:pathology; D003713:Dendritic Cells / Q000276:immunology*; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000276:immunology / Q000473:pathology; D006801:Humans; D007113:Immunity, Innate; D007156:Immunologic Memory; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D009928:Organ Specificity; D019310:Pseudolymphoma / Q000150:complications / Q000276:immunology / Q000473:pathology; D013163:Splenomegaly / Q000150:complications / Q000276:immunology / Q000473:pathology; D018127:Tumor Necrosis Factor Receptor Superfamily, Member 7 / Q000032:analysis
chemical_list: D018127:Tumor Necrosis Factor Receptor Superfamily, Member 7