title: Interleukin-21 overexpression dominates T cell response to Epstein-Barr virus in a fatal case of X-linked lymphoproliferative syndrome type 1.
abstract: Interleukin-21 (IL-21) is a cytokine whose actions are closely related to B cell differentiation into plasma cells as well as to CD8(+) cytolytic T cell effector and memory generation, influencing the T lymphocyte response to different viruses. X-linked lymphoproliferative syndrome type 1 (XLP-1) is a primary immunodeficiency syndrome that is characterized by a high susceptibility to Epstein-Barr virus. We observed in a pediatric patient with XLP-1 that IL-21 was expressed in nearly all peripheral blood CD4(+) and CD8(+) T cells. However, IL-21 could not be found in the lymph nodes, suggesting massive mobilization of activated cells toward the infection's target organs, where IL-21-producing cells were detected, resulting in large areas of tissue damage.
authors: Department of Immunology, School of Medicine, University of Córdoba, Córdoba, Spain.
authors: Consuelo
authors: C
authors: Ortega
authors: Orlando A
authors: OA
authors: Estévez
authors: Silvia
authors: S
authors: Fernández
authors: Rocío
authors: R
authors: Aguado
authors: José M
authors: JM
authors: Rumbao
authors: Teresa
authors: T
authors: Gonzalez
authors: Juan L
authors: JL
authors: Pérez-Navero
authors: Manuel
authors: M
authors: Santamaría
mesh_terms: D015496:CD4-Positive T-Lymphocytes / Q000276:immunology / Q000378:metabolism; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology / Q000378:metabolism; D020031:Epstein-Barr Virus Infections / Q000276:immunology*; D017809:Fatal Outcome; D004854:Herpesvirus 4, Human / Q000276:immunology*; D006801:Humans; D007223:Infant; D007378:Interleukins / Q000096:biosynthesis*; D008198:Lymph Nodes / Q000166:cytology; D008213:Lymphocyte Activation; D008232:Lymphoproliferative Disorders / Q000276:immunology*; D008297:Male; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology*; D000098546:Interleukin-21
chemical_list: D007378:Interleukins; D000098546:Interleukin-21