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

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).

Bibliografische Daten

OriginalspracheEnglisch
ISSN0007-1048
DOIs
StatusVeröffentlicht - 12.2014
PubMed 25209728