title: Thymoma-associated immunodeficiency: a syndrome characterized by severe alterations in NK, T and B-cells and progressive increase in naïve CD8+ T Cells.
abstract: Thymomas are rare tumours that sustain T-lymphopoiesis and trigger a variety of autoimmune diseases and immunodeficiencies, including a fatal hypogammaglobulinemia, namely Goods Syndrome (GS). Due to its rarity, GS has been poorly investigated and immunological features, as well as pathogenetic mechanisms underlying this syndrome, are unclear. We studied 30 thymoma patients by performing an immunological assessment, including immunophenotype and analysis of T cell repertoire (TCR). Development of GS was characterized by a progressive decrease in B, CD4 T and NK lymphocytes. These alterations paired with accumulation of CD8+CD45RA+ T cells that showed a polyclonal repertoire without expansions of specific clonotypes. GS is defined as hypogammaglobulinemia with thymoma. Here, we show for the first time that this syndrome is characterized by a severe loss of CD4+, NK and B cells. Furthermore, the accumulation of CD8+CD45RA+ T lymphocytes parallels these changes; this accumulation may have a role in determining the disease and can be used to monitor clinical stages of immunodeficiency in thymoma.
authors.affiliation: Department of Cellular and Molecular Biology and Pathology, Federico II University of Naples, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Vitiello
authors.forename: A M
authors.initials: AM
authors.lastname: Masci
authors.forename: L
authors.initials: L
authors.lastname: Montella
authors.forename: F
authors.initials: F
authors.lastname: Perna
authors.forename: D F
authors.initials: DF
authors.lastname: Angelini
authors.forename: G
authors.initials: G
authors.lastname: Borsellino
authors.forename: L
authors.initials: L
authors.lastname: Battistini
authors.forename: G
authors.initials: G
authors.lastname: Merola
authors.forename: R
authors.initials: R
authors.lastname: De Palma
authors.forename: G
authors.initials: G
authors.lastname: Spadaro
authors.forename: E
authors.initials: E
authors.lastname: Cosentini
authors.forename: G
authors.initials: G
authors.lastname: Palmieri
authors.forename: L
authors.initials: L
authors.lastname: Racioppi
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000276:immunology*; D000368:Aged; D001402:B-Lymphocytes / Q000276:immunology*; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology*; D022801:Complementarity Determining Regions; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology*; D017493:Leukocyte Common Antigens / Q000032:analysis; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D013945:Thymoma / Q000276:immunology*; D013953:Thymus Neoplasms / Q000276:immunology*
chemical_list: D022801:Complementarity Determining Regions; D017493:Leukocyte Common Antigens