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

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Authors

POSPÍŠILOVÁ Dagmar HOLUB D. ZIDOVA Z. SULOVSKA L. HOUDA J. MIHAL V. HADACOVA I. RADOVÁ Lenka DZUBAK P. HAJDÚCH Marian DIVOKÝ Vladimír HORVATHOVA M.

Year of publication 2014
Type Article in Periodical
Magazine / Source Haematologica
MU Faculty or unit

Central European Institute of Technology

Citation
Web http://www.haematologica.org/content/haematol/99/7/e118.full.pdf
Doi http://dx.doi.org/10.3324/haematol.2014.104034
Field Oncology and hematology
Keywords Diamond Blackfan anemia; hepcidin levels; erythropoietic activity; transfusion dependency
Description Diamond-Blackfan anemia (DBA) is a rare congenital red cell aplasia associated with mutations in ribosomal proteins (RP) in 49-71% of cases.1 DBA is a clinically heterogeneous disorder with one-third of patients developing transfusion-acquired iron overload.2 The severity of anemia and transfusion dependency in DBA is comparable to transfusiondependent beta-thalassemia major; however, moderate to severe suppression of erythropoiesis in DBA2 is in contrast to accelerated ineffective erythropoiesis in beta-thalassemia.3,4 The knowledge of systemic iron regulation in DBA is limited.
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