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. 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.

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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.
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References

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