Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2014 Jul;99(7):e118-21.
doi: 10.3324/haematol.2014.104034. Epub 2014 Apr 11.

Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency

Dagmar Pospisilova  1 , Dusan Holub  2 , Zuzana Zidova  3 , Lucie Sulovska  4 , Jiri Houda  4 , Vladimir Mihal  4 , Ivana Hadacova  5 , Lenka Radova  6 , Petr Dzubak  2 , Marian Hajduch  2 , Vladimir Divoky  3 , Monika Horvathova  7
Affiliations

Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency

Dagmar Pospisilova et al. Haematologica. 2014 Jul.
No abstract available

Keywords: Diamond Blackfan anemia; erythropoietic activity; hepcidin levels; transfusion dependency.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Hepatic iron distribution, hepcidin and GDF15 levels and correlation analyses. (A) Perl’s staining showing iron deposits in the liver of 2 transfusion-dependent DBA patients; CZUH03 at the age of 10 (LIC, 4.6 mg/g d.w.) and CZUH41 at the age of 5 (10.3 mg/g d.w.). Massive iron stores (blue stain) can be found in both hepatocytes and Kupffer cells. Similar pattern of iron staining was obtained for the remaining 3 transfusion-dependent DBA patients analyzed (data not shown). (B) Significantly increased hepcidin levels in comparison with healthy controls were detected for the whole DBA cohort (n=25) and transfusion-dependent DBA patients (TDP, n=12); DBA patients in remission or treated with steroids (RSP, n=11) had hepcidin levels comparable with the controls. (C) GDF15 levels were significantly elevated in the whole DBA cohort (n=25), TDP (n=12) and RSP (n=11) when compared to healthy controls. (D–F) Hepcidin levels showed the following correlations: positive with ferritin (D), negative with the number of erythroblasts in the bone marrow (E) and positive with serum EPO (F). Closed and open circles depict TDP and RSP, respectively. Asterisk shows one patient on steroids and occasional transfusions; the other similarly treated patient (CZUH37) was excluded from statistical analyses due to concomitant occurrence of DBA and HFE C282Y homozygous mutation. The image in (A) was visualized with an Olympus IX 71 light microscope (Olympus, Hamburg, Germany), original magnification 400× and acquired with an Olympus DP 50 camera driven by DP controller software (provided by Olympus). Data in (B and C) are shown in a box plot depicting median (closed square), 25–75% range (box), and the highest and lowest value (the highest and the lowest whiskers, respectively). Image was assembled and labeled using Adobe Photoshop software (Adobe Systems, San Jose, CA, USA). NS: not significant.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Boria I, Garelli E, Gazda HT, Aspesi A, Quarello P, Pavesi E, et al. The ribosomal basis of Diamond-Blackfan Anemia: mutation and database update. Hum Mutat. 2010;31(12):1269–79 - PMC - PubMed
    1. Vlachos A, Ball S, Dahl N, Alter BP, Sheth S, Ramenghi U, et al. Diagnosing and treating Diamond Blackfan anaemia: results of an international clinical consensus conference. Br J Haematol. 2008;142(6):859–76 - PMC - PubMed
    1. Tanno T, Noel P, Miller JL. Growth differentiation factor 15 in erythroid health and disease. Curr Opin Hematol. 2010;17(3):184–90 - PMC - PubMed
    1. Origa R, Galanello R, Ganz T, Giagu N, Maccioni L, Faa G, et al. Liver iron concentrations and urinary hepcidin in beta-thalassemia. Haematologica. 2007;92(5):583–8 - PubMed
    1. Mojzikova R, Koralkova P, Holub D, Zidova Z, Pospisilova D, Cermak J, et al. Iron status in patients with pyruvate kinase deficiency: neonatal hyperferritinaemia associated with a novel frameshift deletion in the PKLR gene (p.Arg518fs), and low hepcidin to ferritin ratios. Br J Haematol. 2014;165(4):556–63 - PubMed

Publication types