title: Defective Fc-, CR1- and CR3-mediated monocyte phagocytosis and chemotaxis in common variable immunodeficiency and X-linked agammaglobulinemia patients.
abstract: Blood monocyte phagocytic functions were evaluated by chemotaxis, phagocytosis, and superoxide anion production in nine patients with common variable immunodeficiency (CVI), eight patients with X-linked agammaglobulinemia (XLA), and in 17 normal subjects. Further laboratory diagnosis included the determination of the Bruton's tyrosine kinase (Btk) protein expression in monocytes using flow cytometry. The analysis of monocyte phagocytic function demonstrated that CR3-, CR1-, and Fc-mediated phagocytosis (p = 0.0001) were significantly decreased in CVI and XLA patients, and chemotaxis of monocytes (p = 0.0082) was reduced in XLA patients. Superoxide anion production, however, did not differ between the CVI, XLA, and the control groups. The cytoplasmic expression of Btk protein in monocytes was normal in CVI patients and decreased or not detected in XLA patients. It is proposed that impaired chemotaxis and phagocytosis by monocytes may be a characteristic of the innate immune system in CVI and XLA patients, providing a new direction for the physiopathology of these immunodeficiencies.
authors.affiliation: Department of Pediatrics, Center for Investigation in Pediatrics-CIPED, School of Medical Sciences, State University of Campinas, Unicamp, Brazil.
authors.forename: A L
authors.initials: AL
authors.lastname: Amoras
authors.forename: H
authors.initials: H
authors.lastname: Kanegane
authors.forename: T
authors.initials: T
authors.lastname: Miyawaki
authors.forename: M M
authors.initials: MM
authors.lastname: Vilela
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000175:diagnosis / Q000235:genetics / Q000276:immunology*; D002478:Cells, Cultured; D002633:Chemotaxis / Q000276:immunology* / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000175:diagnosis / Q000276:immunology*; D005260:Female; D006801:Humans; D016177:Macrophage-1 Antigen / Q000032:analysis / Q000276:immunology*; D008297:Male; D009000:Monocytes / Q000276:immunology / Q000502:physiology; D010587:Phagocytosis / Q000276:immunology* / Q000502:physiology; D011336:Probability; D011505:Protein-Tyrosine Kinases / Q000032:analysis / Q000276:immunology; D017463:Receptors, Complement 3b / Q000032:analysis / Q000276:immunology*; D012494:Sampling Studies; D012680:Sensitivity and Specificity; D018709:Statistics, Nonparametric
chemical_list: D016177:Macrophage-1 Antigen; D017463:Receptors, Complement 3b; D011505:Protein-Tyrosine Kinases