Regulation of iron homeostasis through the erythroferrone-hepcidin axis in sickle cell disease.
erythroferrone
ferritin
hepcidin
iron metabolism
iron overload
sickle cell disease
Journal
British journal of haematology
ISSN: 1365-2141
Titre abrégé: Br J Haematol
Pays: England
ID NLM: 0372544
Informations de publication
Date de publication:
06 2020
06 2020
Historique:
received:
27
10
2019
accepted:
25
12
2019
pubmed:
8
2
2020
medline:
26
1
2021
entrez:
8
2
2020
Statut:
ppublish
Résumé
Sickle cell disease (SCD) has a distinct pattern of transfusional iron overload (IO) when compared to transfusion-dependent β-thalassaemia major (TDT). We conducted a single institution prospective study to evaluate plasma biomarkers of iron regulation and inflammation in patients with SCD with IO (SCD IO cases, n = 22) and without IO (SCD non-IO cases, n = 11), and non-SCD controls (n = 13). Hepcidin was found to be inappropriately low, as evidenced by a significantly higher median hepcidin/ferritin ratio in non-SCD controls compared to SCD IO cases (0·3 vs. 0·02, P < 0·0001) and SCD non-IO cases (0·3 vs. 0·02, P < 0·0001), suggesting that certain inhibitory mechanism (s) work to suppress hepcidin in SCD. As opposed to the SCD non-IO state, where hepcidin shows a strong significant positive correlation with ferritin (Spearman ρ = 0·7, P = 0·02), this correlation was lost when IO occurs (Spearman ρ = -0·2, P = 0·4). Although a direct non-linear correlation between erythroferrone (ERFE) and hepcidin did not reach statistical significance both in the IO (Spearman ρ = -0·4, P = 0·08) and non-IO state (Spearman ρ = -0·6, P = 0·07), patients with highest ERFE had low hepcidin levels, suggesting that ERFE contributes to hepcidin regulation in some patients. Our results suggest a multifactorial mechanism of hepcidin regulation in SCD.
Identifiants
pubmed: 32030737
doi: 10.1111/bjh.16498
pmc: PMC8011855
mid: NIHMS1681534
doi:
Substances chimiques
Erfe protein, human
0
HAMP protein, human
0
Hepcidins
0
Peptide Hormones
0
Ferritins
9007-73-2
Iron
E1UOL152H7
Types de publication
Clinical Trial
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1204-1209Subventions
Organisme : NIDDK NIH HHS
ID : R01 DK065029
Pays : United States
Commentaires et corrections
Type : CommentIn
Informations de copyright
© 2020 British Society for Haematology and John Wiley & Sons Ltd.
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