Common variable immunodeficiency is associated with defective functions of dendritic cells

被引:106
作者
Bayry, J
Lacroix-Desmazes, S
Kazatchkine, MD
Galicier, L
Lepelletier, Y
Webster, D
Lévy, Y
Eibl, MM
Oksenhendler, E
Hermine, O
Kaveri, SV
机构
[1] Inst Biomed Cordeliers, INSERM U430, F-75006 Paris, France
[2] Univ Paris 06, Inst Biomed Cordeliers, F-75006 Paris, France
[3] Hop St Louis, Dept Clin Immunopathol, Paris, France
[4] Hop Necker Enfants Malad, CNRS, UMR 8603, Dept Hematol, Paris, France
[5] Royal Free Med Sch, Dept Immunol, London, England
[6] Hop Henri Mondor, Serv Immunol Clin, F-94010 Creteil, France
[7] Immunol Outpatient Clin, Vienna, Austria
关键词
D O I
10.1182/blood-2004-04-1325
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Common variable immunodeficiency (CVID) is characterized by hypogamma-globulinemia and defects in T-cell functions that could be primary or secondary. We addressed whether CVID is associated with impairment in the dendritic cell (DC) compartment, as DCs play a central role in the development of adaptive immunity. We demonstrate that DCs from CVID patients display severely perturbed differentiation, maturation, and function, and express markedly reduced levels of the costimulatory molecules that are critical for T-cell stimulation. Patients' DCs induced weak proliferation of allogeneic T cells and produced significantly low amounts of interleukin-12 (IL-12) upon CD40 signaling. Multiple defects in the immune system, including malfunctioning of DCs, appear to be prominent features of CVID patients. Impairment in both the innate and adaptive compartments of the immune system may thus cumulatively account for the inability of CVID patients to eradicate pathogens through conventional immune pathways, thus resulting in an increased risk for recurrent bacterial infections. (C) 2004 by The American Society of Hematology.
引用
收藏
页码:2441 / 2443
页数:3
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