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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
Author(s) -
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
Publication year - 2014
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.13081
Subject(s) - hepcidin , erythropoiesis , transferrin , ineffective erythropoiesis , diamond–blackfan anemia , medicine , transferrin receptor , immunology , erythropoietin , ferroportin , anemia , endocrinology , chemistry , biochemistry , ribosome , rna , gene
Summary 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 SLC 40A1 [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 ).