The role of Treg cells and FoxP3 expression in immunity of β-thalassemia major AND β-thalassemia trait patients

Pediatr Hematol Oncol. 2010 Oct;27(7):534-45. doi: 10.3109/08880018.2010.503334.

Abstract

There is increased susceptibility to infections in β-thalassemia. Changes in T- and B-lymphocyte subsets and functions, defective chemotaxis, and phagocytosis of neutrophils and macrophages have been described in these patients. Regulatory T cells (Treg cells) play a crucial role in the maintenance of immunological self-tolerance. The FOXP3 gene is specifically expressed on Treg cells. Increased antigenic stimuli due to repeated blood transfusions might change the Treg cells and FOXP3 percentage in β-thalassemia. Immune functions of peripheral blood lymphocytes, percentage of Treg cells (defined as CD4(+)CD25(+)FoxP3(+)) were evaluated in 30 β-thalassemia major, 30 β-thalassemia trait, and 20 healthy children. Percentage of CD4(+)CD45RA(+) cells were increased in β-thalassemia trait compared to both β-thalassemia major and controls, whereas percentage of CD4(+)CD45RO(+) cells were higher in β-thalassemia major and trait patient compared to controls. Percentages of CD4(+)CD25(bright) and CD4(+)CD25(+)FoxP3(+) Treg cells were increased only in β-thalassemia major patients compared to controls (P = .001 and P = .0001, respectively). T lymphocytes express activated phenotype both in β-thalassemia major and trait patients. However, only in β-thalassemia major patients who were exposed to chronic antigenic stimulus as a result of repeated blood transfusions was an increase observed in Treg cells, which might suppress immune activation status.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Female
  • Follow-Up Studies
  • Forkhead Transcription Factors / biosynthesis*
  • Forkhead Transcription Factors / blood
  • Forkhead Transcription Factors / immunology
  • Humans
  • Immunity*
  • Infant
  • Male
  • T-Lymphocyte Subsets / immunology
  • T-Lymphocytes, Helper-Inducer / immunology
  • T-Lymphocytes, Regulatory / immunology*
  • Young Adult
  • beta-Thalassemia / blood
  • beta-Thalassemia / immunology*

Substances

  • FOXP3 protein, human
  • Forkhead Transcription Factors