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

Varování

Publikace nespadá pod Ústav výpočetní techniky, ale pod Středoevropský technologický institut. Oficiální stránka publikace je na webu muni.cz.

Autoři	POSPÍŠILOVÁ Dagmar HOLUB D. ZIDOVA Z. SULOVSKA L. HOUDA J. MIHAL V. HADACOVA I. RADOVÁ Lenka DZUBAK P. HAJDÚCH Marian DIVOKÝ Vladimír HORVATHOVA M.
Rok publikování	2014
Druh	Článek v odborném periodiku
Časopis / Zdroj	Haematologica
Fakulta / Pracoviště MU	Středoevropský technologický institut
Citace
www	http://www.haematologica.org/content/haematol/99/7/e118.full.pdf
Doi	https://doi.org/10.3324/haematol.2014.104034
Obor	Onkologie a hematologie
Klíčová slova	Diamond Blackfan anemia; hepcidin levels; erythropoietic activity; transfusion dependency
Popis	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.
Související projekty:	NGS-PTL - Next Generation Sequencing platform for targeted Personalized Therapy of Leukemia