Loading…

Thymoma-Associated Immunodeficiency: A Syndrome Characterized by Severe Alterations in NK, T and B-Cells and Progressive Increase in Naïve CD8+ T Cells

Thymomas are rare tumours that sustain T-lymphopoiesis and trigger a variety of autoimmune diseases and immunodeficiencies, including a fatal hypogammaglobulinemia, namely Good's Syndrome (GS). Due to its rarity, GS has been poorly investigated and immunological features, as well as pathogeneti...

Full description

Saved in:
Bibliographic Details
Published in:International journal of immunopathology and pharmacology 2010-01, Vol.23 (1), p.307-316
Main Authors: Vitiello, L., Masci, A.M., Montella, L., Perna, F., Angelini, D.F., Borsellino, G., Battistini, L., Merola, G., De Palma, R., Spadaro, G., Cosentini, E., Palmieri, G., Racioppi, L.
Format: Article
Language:English
Subjects:
Citations: Items that this one cites
Items that cite this one
Online Access:Request full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Thymomas are rare tumours that sustain T-lymphopoiesis and trigger a variety of autoimmune diseases and immunodeficiencies, including a fatal hypogammaglobulinemia, namely Good's 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.
ISSN:0394-6320
2058-7384
DOI:10.1177/039463201002300129