Literature DB >> 25209728

Mechanisms of plasma non-transferrin bound iron generation: insights from comparing transfused diamond blackfan anaemia with sickle cell and thalassaemia patients.

John B Porter1, Patrick B Walter, Lynne D Neumayr, Patricia Evans, Sukhvinder Bansal, Maciej Garbowski, Marcela G Weyhmiller, Paul R Harmatz, John C Wood, Jeffery L Miller, Colleen Byrnes, Guenter Weiss, Markus Seifert, Regine Grosse, Dagmar Grabowski, Angelica Schmidt, Roland Fischer, Peter Nielsen, Charlotte Niemeyer, Elliott Vichinsky.   

Abstract

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).
© 2014 John Wiley & Sons Ltd.

Entities:  

Keywords:  NTBI; erythropoiesis; hepcidin; inflammation; iron overload; non-transferrin bound iron

Mesh：

Substances：

Year:  2014        PMID： 25209728      PMCID： PMC4577015          DOI： 10.1111/bjh.13081

Source DB:  PubMed          Journal:  Br J Haematol        ISSN： 0007-1048            Impact factor:   6.998


  15 in total

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