Authors: Bönnemann, Vivian
Claus, Maren
Butzeck, Barbara
Collette, Daniela
Bröde, Peter
Golka, Klaus
Watzl, Carsten
Title: Analysis of natural killer cell functions in patients with hereditary hemochromatosis
Language (ISO): en
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.
Subject Headings: Hereditary hemochromatosis
Immune System
Natural Killer Cells
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Issue Date: 2020-03-25
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Provenance: IfADo - Leibniz Research Centre for Working Environment and Human Factors, Dortmund
Citation: 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.
Appears in Collections:Original Articles 2020

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