Analysis of Natural Killer cell functions in patients with hereditary hemochromatosis

Authors

  • Vivian Bönnemann Department for Immunology, Leibniz Research Centre for Working Environment and Human Factors (IfADo) at TU Dortmund, Dortmund, Germany
  • Maren Claus Department for Immunology, Leibniz Research Centre for Working Environment and Human Factors (IfADo) at TU Dortmund, Dortmund, Germany
  • Barbara Butzeck Hämochromatose-Vereinigung Deutschland e.V. HVD, European Federation of Associations of Patients with Haemochromatosis EFAPH, Hattingen, Germany
  • Daniela Collette Gemeinschaftspraxis für Hämatologie und Onkologie, Dortmund, Germany
  • Peter Bröde Department for Immunology, Leibniz Research Centre for Working Environment and Human Factors (IfADo) at TU Dortmund, Dortmund, Germany
  • Klaus Golka Department for Immunology, Leibniz Research Centre for Working Environment and Human Factors (IfADo) at TU Dortmund, Dortmund, Germany
  • Carsten Watzl Leibniz Research Centre for Working Environment and Human Factors (IfADo) at TU Dortmund, Ardeystrasse 67, 44139 Dortmund, Germany, E-mail: watzl@ifado.de http://orcid.org/0000-0001-5195-0995

DOI:

https://doi.org/10.17179/excli2020-1116

Keywords:

Hereditary hemochromatosis, Immune System, iron, Natural Killer Cells

Abstract

Hereditary hemochromatosis (HH) is an autosomal-recessive disorder of the iron metabolism. Patients are typically affected by dysregulated iron levels, which can lead to iron accumulation within essential organs, such as liver, heart and pancreas. Furthermore, many HH patients are also afflicted by several immune defects and increased occurrence of autoimmune diseases that are linked to human homeostatic iron regulator protein (HFE) in the immune response. Here we examined immune cell phenotype and function in 21 HH patients compared to 21 healthy controls with a focus on Natural Killer (NK) cells. We observed increased basal and stimulated production of pro-inflammatory cytokines such as IL-1β or IL-18 in HH patients compared to healthy controls. However, we did not find major changes in the phenotype, the amount or the cytotoxic function of NK cells in HH patients. Instead, our data show a general decrease in the total number of granulocytes in HH patients (2774 ± 958 per μl versus 3457 ± 1122 per μl in healthy controls). These data demonstrate that NK cells of HH patients are not significantly affected and that the patients’ treatment by regular phlebotomy is sufficient to avoid systemic iron overload and its consequences to the immune system.

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Published

2020-03-25

How to Cite

Bönnemann, V., Claus, M., Butzeck, B., Collette, D., Bröde, P., Golka, K., & Watzl, C. (2020). Analysis of Natural Killer cell functions in patients with hereditary hemochromatosis. EXCLI Journal, 19, 430–441. https://doi.org/10.17179/excli2020-1116

Issue

Vol. 19 (2020)

Section

Original articles

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