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

L. Vitiello, A. M. Masci, L. Montella, F. Perna, D. F. Angelini, G. Borsellino, L. Battistini, G. Merola, R. De Palma, G. Spadaro, E. Cosentini, G. Palmieri, L. Racioppi

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)307-316
Number of pages10
JournalInternational Journal of Immunopathology and Pharmacology
Volume23
Issue number1
Publication statusPublished - Jan 2010

Keywords

  • B lymphocytes
  • CD8
  • Good's syndrome
  • Immunodeficiency
  • NK
  • Thymoma

ASJC Scopus subject areas

  • Pharmacology
  • Immunology
  • Immunology and Allergy

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