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

John B Porter, 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

Journal: British journal of haematology 2015;167(5):692-6

PMID: 25209728

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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).

Address: Department of Haematology, University College London, London, UK.

DOI: 10.1111/bjh.13081

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MeSH Terms: Adolescent, Adult, Anemia, Diamond-Blackfan, Anemia, Sickle Cell, Biomarkers, Blood Proteins, Blood Transfusion, Erythropoiesis, Female, Humans, Iron, Iron Overload, Male, Thalassemia, Transferrin