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High levels of soluble serum hemojuvelin in patients with congenital dyserythropoietic anemia type I.
MedLine Citation:
PMID:  23095116     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
OBJECTIVE: Congenital dyserythropoietic anemia (CDA) is a rare group of red blood cell disorders with ineffective erythropoiesis and secondary hemochromatosis. Inappropriate suppression of hepcidin and high levels of growth differentiation factor 15 (GDF15) have been described in CDA I and II patients, probably contributing to secondary hemochromatosis. Hemojuvelin (HJV) is an important regulator of serum hepcidin while soluble form of HJV (s-HJV) competitively down-regulates hepcidin. METHODS: We determined the soluble hemojuvelin (s-HJV) levels in 17 patients with CDA I and in 17 healthy volunteers (HV) and looked for correlations with other parameters of iron overload and erythropoiesis. RESULTS: Significantly higher levels of s-HJV were found in patients (2.32±1.40 mg/L) compared to healthy volunteers (0. 69±0.44 mg/L) (p= 0.001). Western blot analysis confirmed the presence of high levels of s-HJV in CDA I patients. s-HJV positively correlated with serum ferritin, erythropoietin, soluble transferrin receptor, GDF-15, and negatively correlated with hepcidin to ferritin ratios. CONCLUSIONS: We for the first time documented high levels of serum s-HJV in CDA I patients suggesting that it may contribute to iron loading pathology in CDA I and eventually in other anemias with ineffective erythropoiesis. © 2012 John Wiley & Sons A/S.
Authors:
Hanna Shalev; Galit Perez-Avraham; Joseph Kapelushnik; Itai Levi; Anat Rabinovich; Dorine W Swinkels; Carole Brasse-Lagnel; Hannah Tamary
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-10-24
Journal Detail:
Title:  European journal of haematology     Volume:  -     ISSN:  1600-0609     ISO Abbreviation:  Eur. J. Haematol.     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-25     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8703985     Medline TA:  Eur J Haematol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2012 John Wiley & Sons A/S.
Affiliation:
Hematology Department Soroka Medical Center, Faculty of Medicine, Ben-Gurion University, Beer Sheva, Israel.
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