Mechanisms of plasma non-transferrin bound iron generation: insights from comparing transfused diamond blackfan anaemia with sickle cell and thalassaemia patients
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Mechanisms of plasma non-transferrin bound iron generation: insights from comparing transfused diamond blackfan anaemia with sickle cell and thalassaemia patients. / Porter, John B; Walter, Patrick B; Neumayr, Lynne D; Evans, Patricia; Bansal, Sukhvinder; Garbowski, Maciej; Weyhmiller, Marcela G; Harmatz, Paul R; Wood, John C; Miller, Jeffery L; Byrnes, Colleen; Weiss, Guenter; Seifert, Markus; Grosse, Regine; Grabowski, Dagmar; Schmidt, Angelica; Fischer, Roland; Nielsen, Peter; Niemeyer, Charlotte; Vichinsky, Elliott.
In: BRIT J HAEMATOL, Vol. 167, No. 5, 12.2014, p. 692-6.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Mechanisms of plasma non-transferrin bound iron generation: insights from comparing transfused diamond blackfan anaemia with sickle cell and thalassaemia patients
AU - Porter, John B
AU - Walter, Patrick B
AU - Neumayr, Lynne D
AU - Evans, Patricia
AU - Bansal, Sukhvinder
AU - Garbowski, Maciej
AU - Weyhmiller, Marcela G
AU - Harmatz, Paul R
AU - Wood, John C
AU - Miller, Jeffery L
AU - Byrnes, Colleen
AU - Weiss, Guenter
AU - Seifert, Markus
AU - Grosse, Regine
AU - Grabowski, Dagmar
AU - Schmidt, Angelica
AU - Fischer, Roland
AU - Nielsen, Peter
AU - Niemeyer, Charlotte
AU - Vichinsky, Elliott
N1 - © 2014 John Wiley & Sons Ltd.
PY - 2014/12
Y1 - 2014/12
N2 - In transfusional iron overload, extra-hepatic iron distribution differs, depending on the underlying condition. Relative mechanisms of plasma non-transferrin bound iron (NTBI) generation may account for these differences. Markers of iron metabolism (plasma NTBI, labile iron, hepcidin, transferrin, monocyte SLC40A1 [ferroportin]), erythropoiesis (growth differentiation factor 15, soluble transferrin receptor) and tissue hypoxia (erythropoietin) were compared in patients with Thalassaemia Major (TM), Sickle Cell Disease and Diamond-Blackfan Anaemia (DBA), with matched transfusion histories. The most striking differences between these conditions were relationships of NTBI to erythropoietic markers, leading us to propose three mechanisms of NTBI generation: iron overload (all), ineffective erythropoiesis (predominantly TM) and low transferrin-iron utilization (DBA).
AB - In transfusional iron overload, extra-hepatic iron distribution differs, depending on the underlying condition. Relative mechanisms of plasma non-transferrin bound iron (NTBI) generation may account for these differences. Markers of iron metabolism (plasma NTBI, labile iron, hepcidin, transferrin, monocyte SLC40A1 [ferroportin]), erythropoiesis (growth differentiation factor 15, soluble transferrin receptor) and tissue hypoxia (erythropoietin) were compared in patients with Thalassaemia Major (TM), Sickle Cell Disease and Diamond-Blackfan Anaemia (DBA), with matched transfusion histories. The most striking differences between these conditions were relationships of NTBI to erythropoietic markers, leading us to propose three mechanisms of NTBI generation: iron overload (all), ineffective erythropoiesis (predominantly TM) and low transferrin-iron utilization (DBA).
KW - Adolescent
KW - Adult
KW - Anemia, Diamond-Blackfan
KW - Anemia, Sickle Cell
KW - Biomarkers
KW - Blood Proteins
KW - Blood Transfusion
KW - Erythropoiesis
KW - Female
KW - Humans
KW - Iron
KW - Iron Overload
KW - Male
KW - Thalassemia
KW - Transferrin
U2 - 10.1111/bjh.13081
DO - 10.1111/bjh.13081
M3 - SCORING: Journal article
C2 - 25209728
VL - 167
SP - 692
EP - 696
JO - BRIT J HAEMATOL
JF - BRIT J HAEMATOL
SN - 0007-1048
IS - 5
ER -