Iron restriction in sickle cell disease: When less is more

Castro, OL; De Franceschi, L; Ganz, T; Kanter, J; Kato, GJ; Pasricha, SR; Rivella, S; Wood, JC

Author(s): Castro, OL; De Franceschi, L; Ganz, T; Kanter, J; Kato, GJ; Pasricha, SR; Rivella, S; Wood, JC;
Details: Publication Year 2024-02-23,Volume 99,Issue #7,Page 1349-1359
Journal Title: American Journal of Hematology
Abstract: Primum non nocere! Can iron deficiency, an abnormality that causes anemia, benefit people with sickle cell disease (SCD) who already have an anemia? The published literature we review appears to answer this question in the affirmative: basic science considerations, animal model experiments, and noncontrolled clinical observations all suggest a therapeutic potential of iron restriction in SCD. This is because SCD's clinical manifestations are ultimately attributable to the polymerization of hemoglobin S (HbS), a process strongly influenced by intracellular HbS concentration. Even small decrements in HbS concentration greatly reduce polymerization, and iron deficiency lowers erythrocyte hemoglobin concentration. Thus, iron deficiency could improve SCD by changing its clinical features to those of a more benign anemia (i.e., a condition with fewer or no vaso-occlusive events). We propose that well-designed clinical studies be implemented to definitively determine whether iron restriction is a safe and effective option in SCD. These investigations are particularly timely now that pharmacologic agents are being developed, which may directly reduce red cell hemoglobin concentrations without the need for phlebotomies to deplete total body iron.
Publisher: Wiley
Keywords: *Anemia, Sickle Cell/complications/blood; Humans; Animals; *Iron/metabolism/blood; *Hemoglobin, Sickle/metabolism/analysis; Anemia, Iron-Deficiency/drug therapy; Erythrocytes/metabolism
Research Division(s): Population Health And Immunity
PubMed ID: 38400590
Publisher's Version: https://doi.org/10.1002/ajh.27267
Open Access at Publisher's Site: https://doi.org/10.1002/ajh.27267
Terms of Use/Rights Notice: Refer to copyright notice on published article.

Creation Date: 2024-02-29 09:16:07

Last Modified: 2024-06-28 03:08:42