title: Defective functions of polymorphonuclear neutrophils in patients with common variable immunodeficiency.
abstract: Common variable immunodeficiency (CVID) is a heterogeneous antibody deficiency condition with alterations in T cell regulation and function, dendritic and B-cell compartment and represents the most frequent cause of symptomatic primary immunodeficiency. We addressed whether CVID is associated with abnormalities in the polymorphonuclear neutrophil (PMN) compartment, an important component of innate immunity and plays a key role in host defenses against invading microorganisms. We used flow cytometry to examine PMN phenotypic and functional abnormalities in CVID patients, using whole-blood conditions in order to avoid artifacts due to isolation procedures. We demonstrated that PMN from CVID patients displays, at resting state, a decreased expression of CD15, CD11b and CD16b, which might be related to an abnormality in neutrophil maturation. In addition, these neutrophils exhibit a decrease in degranulation, phagocytosis and reactive oxygen species production, as well as an increased death by apoptosis. These PMN abnormalities observed in CVID patients could result in an increased risk for recurrent bacterial infections.
authors: UMR-S CR7, Sorbonne University, UPMC University Paris 06, 75005, Paris, France.
authors: Sarah
authors: S
authors: Casulli
authors: Hélène
authors: H
authors: Coignard-Biehler
authors: Karima
authors: K
authors: Amazzough
authors: Michka
authors: M
authors: Shoai-Tehrani
authors: Jagadeesh
authors: J
authors: Bayry
authors: Nizar
authors: N
authors: Mahlaoui
authors: Carole
authors: C
authors: Elbim
authors: Srini V
authors: SV
authors: Kaveri
mesh_terms: D000328:Adult; D000368:Aged; D017209:Apoptosis; D015550:Cell Degranulation; D017074:Common Variable Immunodeficiency / Q000276:immunology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007958:Leukocyte Count; D008297:Male; D008875:Middle Aged; D009504:Neutrophils / Q000502:physiology*; D010587:Phagocytosis; D010641:Phenotype; D017382:Reactive Oxygen Species / Q000378:metabolism
chemical_list: D017382:Reactive Oxygen Species