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

J 2014

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

POSPÍŠILOVÁ, Dagmar, D. HOLUB, Z. ZIDOVA, L. SULOVSKA, J. HOUDA et. al.

Basic information

Original name

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

Authors

POSPÍŠILOVÁ, Dagmar (203 Czech Republic), D. HOLUB (203 Czech Republic), Z. ZIDOVA (203 Czech Republic), L. SULOVSKA (203 Czech Republic), J. HOUDA (203 Czech Republic), V. MIHAL (203 Czech Republic), I. HADACOVA (203 Czech Republic), Lenka RADOVÁ (203 Czech Republic, guarantor, belonging to the institution), P. DZUBAK (203 Czech Republic), Marian HAJDÚCH (203 Czech Republic), Vladimír DIVOKÝ (203 Czech Republic) and M. HORVATHOVA (203 Czech Republic)

Edition

Haematologica, PAVIA, FERRATA STORTI FOUNDATION, 2014, 0390-6078

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30200 3.2 Clinical medicine

Country of publisher

Italy

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 5.814

RIV identification code

RIV/00216224:14740/14:00078458

Organization unit

Central European Institute of Technology

DOI

http://dx.doi.org/10.3324/haematol.2014.104034

UT WoS

000342833500008

Keywords in English

Diamond Blackfan anemia; hepcidin levels; erythropoietic activity; transfusion dependency

Abstract

V originále

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.

Links

306242, interní kód MU

Name: NGS-PTL - Next Generation Sequencing platform for targeted Personalized Therapy of Leukemia (Acronym: NGS-PTL)

Investor: European Union, Cooperation

Citovat

POSPÍŠILOVÁ, Dagmar, D. HOLUB, Z. ZIDOVA, L. SULOVSKA, J. HOUDA, V. MIHAL, I. HADACOVA, Lenka RADOVÁ, P. DZUBAK, Marian HAJDÚCH, Vladimír DIVOKÝ and M. HORVATHOVA. Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency. Haematologica. PAVIA: FERRATA STORTI FOUNDATION, 2014, vol. 99, No 7, p. 118-121. ISSN 0390-6078. Available from: https://dx.doi.org/10.3324/haematol.2014.104034.

@article{1217008,
   author = {Pospíšilová, Dagmar and Holub, D. and Zidova, Z. and Sulovska, L. and Houda, J. and Mihal, V. and Hadacova, I. and Radová, Lenka and Dzubak, P. and Hajdúch, Marian and Divoký, Vladimír and Horvathova, M.},
   article_location = {PAVIA},
   article_number = {7},
   doi = {http://dx.doi.org/10.3324/haematol.2014.104034},
   keywords = {Diamond Blackfan anemia; hepcidin levels; erythropoietic activity; transfusion dependency},
   language = {eng},
   issn = {0390-6078},
   journal = {Haematologica},
   title = {Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency},
   url = {http://www.haematologica.org/content/haematol/99/7/e118.full.pdf},
   volume = {99},
   year = {2014}
}

TY  - JOUR
ID  - 1217008
AU  - Pospíšilová, Dagmar - Holub, D. - Zidova, Z. - Sulovska, L. - Houda, J. - Mihal, V. - Hadacova, I. - Radová, Lenka - Dzubak, P. - Hajdúch, Marian - Divoký, Vladimír - Horvathova, M.
PY  - 2014
TI  - Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency
JF  - Haematologica
VL  - 99
IS  - 7
SP  - 118-121
EP  - 118-121
PB  - FERRATA STORTI FOUNDATION
SN  - 03906078
KW  - Diamond Blackfan anemia
KW  - hepcidin levels
KW  - erythropoietic activity
KW  - transfusion dependency
UR  - http://www.haematologica.org/content/haematol/99/7/e118.full.pdf
L2  - http://www.haematologica.org/content/haematol/99/7/e118.full.pdf
N2  - 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.
ER  -

POSPÍŠILOVÁ, Dagmar, D. HOLUB, Z. ZIDOVA, L. SULOVSKA, J. HOUDA, V. MIHAL, I. HADACOVA, Lenka RADOVÁ, P. DZUBAK, Marian HAJDÚCH, Vladimír DIVOKÝ and M. HORVATHOVA. Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency. \textit{Haematologica}. PAVIA: FERRATA STORTI FOUNDATION, 2014, vol.~99, No~7, p.~118-121. ISSN~0390-6078. Available from: https://dx.doi.org/10.3324/haematol.2014.104034.

Detailed Information on Publication Record