Thymoma-associated immunodeficiency: a syndrome characterized by severe alterations in NK, T and B-cells and progressive increase in naïve CD8+ T Cells.

Published

Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Vitiello, L; Masci, AM; Montella, L; Perna, F; Angelini, DF; Borsellino, G; Battistini, L; Merola, G; De Palma, R; Spadaro, G; Cosentini, E; Palmieri, G; Racioppi, L

Published Date

  • January 2010

Published In

Volume / Issue

  • 23 / 1

Start / End Page

  • 307 - 316

PubMed ID

  • 20378017

Pubmed Central ID

  • 20378017

Electronic International Standard Serial Number (EISSN)

  • 2058-7384

International Standard Serial Number (ISSN)

  • 0394-6320

Digital Object Identifier (DOI)

  • 10.1177/039463201002300129

Language

  • eng