AI Article Synopsis

  • Hereditary hemochromatosis (HH) is an inherited condition that causes iron overload in vital organs, leading to various health issues and is also associated with immune system defects.
  • A study compared the immune cell function of 21 HH patients to 21 healthy individuals, focusing on Natural Killer (NK) cells, finding increased production of pro-inflammatory cytokines in HH patients.
  • Despite the elevated cytokine levels, NK cell characteristics remained largely unchanged, and there was a decrease in granulocyte numbers, indicating that regular blood removal treatments for HH are effective in managing the condition without severely impacting NK cell function.

Article 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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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7174573PMC
http://dx.doi.org/10.17179/excli2020-1116DOI Listing

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