title: Dendritic and T cell response to influenza is normal in the patients with X-linked agammaglobulinemia.
abstract: BACKGROUND
  Influenza virus is a potential cause of severe disease in the immunocompromised. X-linked agammaglobulinemia(XLA) is a primary immunodeficiency characterized by the lack of immunoglobulin, B cells, and plasma cells,secondary to mutation in Bruton’s tyrosine kinase (Btk) gene. Btk is expressed in both B and dendritic cells (DC). However, little is known about the immune response of DC and T cells to influenza virus in XLA patients.
  
  
  METHODS
  The in vitro maturation and antigen presenting function of monocyte-derived immature DC (im DC) from 12 XLA patients and 23 age-matched normal controls in response to influenza virus were examined. Influenza virus specific CD4 and CD8 T cell responses in the patients and controls were further determined after administration of inactivated trivalent influenza vaccine.
  
  
  RESULTS
  im DC from XLA patients had normal maturation based on major histocompatibility complex (MHC)-I, MHCII, CD83 and CD86 expression, and interferon (IFN)-α and interleukin-12 production upon influenza virus stimulation.They also had a normal capacity to induce allogeneic T cell proliferation in response to influenza virus. TIV was well tolerated in XLA patients. Influenza virus-specific CD4+IFN-γ+ and CD8+ IFN-γ+ T cells and HLA-A2/M158–66-tetramer+ CTLs could be induced by TIV in XLA patients, and the levels and duration of maintaining these virus-specific cells in XLA patients are comparable to that in normal controls.
  
  
  CONCLUSIONS
  We demonstrated for the first time that XLA patients have fully competent DC and T cell immune responses to influenza virus. TIV is safe and could be an option for providing T cell-mediated protection against influenza virus infection in XLA patients.
authors.affiliation: Department of Paediatrics & Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Room L7-58, 7/F Laboratory Block, Faculty of Medicine Building, 21 Sassoon Road, Pokfulam, Hong Kong, China.
authors.forename: Yinping
authors.initials: Y
authors.lastname: Liu
authors.forename: Yuet
authors.initials: Y
authors.lastname: Wu
authors.forename: Kwok-Tai
authors.initials: KT
authors.lastname: Lam
authors.forename: Pamela Pui-Wah
authors.initials: PP
authors.lastname: Lee
authors.forename: Wenwei
authors.initials: W
authors.lastname: Tu
authors.forename: Yu-Lung
authors.initials: YL
authors.lastname: Lau
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000276:immunology*; D016022:Case-Control Studies; D002454:Cell Differentiation; D049109:Cell Proliferation; D003713:Dendritic Cells / Q000166:cytology / Q000276:immunology*; D040181:Genetic Diseases, X-Linked / Q000276:immunology*; D006801:Humans; D007252:Influenza Vaccines / Q000008:administration & dosage*; D007251:Influenza, Human / Q000276:immunology / Q000517:prevention & control; D016898:Interferon-alpha / Q000276:immunology; D018664:Interleukin-12 / Q000276:immunology; D013601:T-Lymphocytes / Q000166:cytology / Q000276:immunology*; D055815:Young Adult
chemical_list: D007252:Influenza Vaccines; D016898:Interferon-alpha; D018664:Interleukin-12