Published online 16 July 2009
Haematologica, Vol 94, Issue 9, 1297-1300 doi:10.3324/haematol.2009.005975
Copyright © 2009 by Ferrata Storti Foundation
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Google Scholar
Right arrow Articles by van Deuren, M.
Right arrow Articles by Swinkels, D. W.
PubMed
Right arrow PubMed Citation
Right arrow Articles by van Deuren, M.
Right arrow Articles by Swinkels, D. W.
Related Collections
Right arrowRelated Article

Disorders of Iron Metabolism

Time-course analysis of serum hepcidin, iron and cytokines in a C282Y homozygous patient with Schnitzler’s syndrome treated with IL-1 receptor antagonist

Marcel van Deuren1, Joyce J. C. Kroot2, Dorine W. Swinkels2

1 Department of General Internal Medicine
2 Department of Clinical Chemistry, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands

Correspondence: Dorine W. Swinkels, MD, PhD, Department of Clinical Chemistry, 441 Radboud University, Nijmegen Medical Center, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands. E-mail:d.swinkels{at}akc.umcn.nl

It is currently unknown if the increase of the hepatic iron regulatory hormone hepcidin during inflammation in man depends on an intact HFE-protein. Here we describe the temporal relationship of serum hepcidin, serum iron and cytokines in a patient with HFE-related (C282Y homozygous) hereditary hemochromatosis who was treated for an auto-inflammatory condition, i.e. variant Schnitzler’s syndrome, with the potent anti-inflammatory cytokine inter-leukin-1 receptor antagonist (IL-1ra, anakinra). The patient had bouts of fever with peaking serum IL-6 concentrations followed by peaking serum hepcidin levels, while serum iron was low. Upon treatment, these peaks disappeared and hepcidin levels became non-detectable, consistent with HFE deficiency. In conclusion, this in vivo human model: i) supports the importance of an HFE-independent IL-6-hepcidin axis in the development of hypoferremia and anemia of inflammation; and ii) suggests that chronic inflammation protects patients with HFE-related hereditary hemochromatosis from iron accumulation.

Key words: hepcidin, iron, hemochromatosis, inflammation, interleukin-1.


Related Article

Genetic variation in hepcidin expression and its implications for phenotypic differences in iron metabolism
Henry K. Bayele, Surjit Kaila S. Srai
Haematologica 2009 94: 1185-1188. [Full Text] [PDF]



This article has been cited by other articles:


Home page
 Circ Cardiovasc GenetHome page
J. L. Sullivan
Do Hemochromatosis Mutations Protect Against Iron-Mediated Atherogenesis?
Circ Cardiovasc Genet, December 1, 2009; 2(6): 652 - 657.
[Full Text] [PDF]

Home page
 haematolHome page
H. K. Bayele and S. K. S. Srai
Genetic variation in hepcidin expression and its implications for phenotypic differences in iron metabolism
Haematologica, September 1, 2009; 94(9): 1185 - 1188.
[Full Text] [PDF]