Immune regulation in chronically transfused allo-antibody responder and nonresponder patients with sickle cell disease and β-thalassemia major

Am J Hematol. 2011 Dec;86(12):1001-6. doi: 10.1002/ajh.22167. Epub 2011 Sep 26.

Abstract

Red blood cell alloimmunization is a major complication of transfusion therapy. Host immune markers that can predict antibody responders remain poorly described. As regulatory T cells (Tregs) play a role in alloimmunization in mouse models, we analyzed the Treg compartment of a cohort of chronically transfused patients with sickle cell disease (SCD, n = 22) and β-thalassemia major (n = 8) with and without alloantibodies. We found reduced Treg activity in alloantibody responders compared with nonresponders as seen in mice. Higher circulating anti-inflammatory IL-10 levels and lower IFN-γ levels were detected in non-alloimmunized SCD patients. Stimulated sorted CD4+ cells from half of the alloimmunized patients had increased frequency of IL-4 expression compared with nonresponders, indicating a skewed T helper (Th) 2 humoral immune response in a subgroup of antibody responders. All patients had increased Th17 responses, suggesting an underlying inflammatory state. Although small, our study indicates an altered immunoregulatory state in alloantibody responders which may help future identification of potential molecular risk factors for alloimmunization.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Adult
  • Biomarkers / blood
  • Biomarkers / metabolism
  • Cell Proliferation
  • Cells, Cultured
  • Coculture Techniques
  • Cohort Studies
  • Female
  • Hemoglobin SC Disease / blood
  • Hemoglobin SC Disease / immunology*
  • Hemoglobin SC Disease / metabolism
  • Hemoglobin SC Disease / therapy
  • Homozygote
  • Humans
  • Immunomodulation*
  • Interferon-gamma / blood
  • Interferon-gamma / metabolism
  • Interleukins / blood
  • Interleukins / metabolism
  • Isoantibodies / analysis
  • Isoantigens / adverse effects*
  • Male
  • T-Lymphocytes, Regulatory / immunology*
  • T-Lymphocytes, Regulatory / metabolism
  • Th2 Cells / immunology*
  • Th2 Cells / metabolism
  • Transfusion Reaction*
  • Young Adult
  • beta-Thalassemia / blood
  • beta-Thalassemia / immunology*
  • beta-Thalassemia / metabolism
  • beta-Thalassemia / therapy

Substances

  • Biomarkers
  • Interleukins
  • Isoantibodies
  • Isoantigens
  • Interferon-gamma