Iron restriction in sickle cell disease: When less is more.

Autor: Castro OL; Center for Sickle Cell Disease, Howard University, Washington, District of Columbia, USA., De Franceschi L; Department of Medicine, University of Verona and AOUI Verona, Verona, Italy., Ganz T; Center for Iron Disorders, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California, USA., Kanter J; Division of Hematology and Oncology, University of Alabama, Birmingham, Birmingham, Alabama, USA., Kato GJ; Hematology Therapeutic Area, CSL Behring, King of Prussia, Pennsylvania, USA., Pasricha SR; Population Health and Immunity Division, Walter and Eliza Hall Institute of Medical Research, Parkville, Victoria, Australia., Rivella S; Division of Hematology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA; RNA Institute, University of Pennsylvania, Philadelphia, Pennsylvania, USA.; RNA Gene Therapeutics Group, RNA Institute, University of Pennsylvania, Philadelphia, Pennsylvania, USA., Wood JC; Division of Cardiology, Department of Pediatrics and Radiology, Children's Hospital of Los Angeles, Los Angeles, California, USA.
Jazyk: angličtina
Zdroj: American journal of hematology [Am J Hematol] 2024 Jul; Vol. 99 (7), pp. 1349-1359. Date of Electronic Publication: 2024 Feb 23.
DOI: 10.1002/ajh.27267
Abstrakt: 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.
(© 2024 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.)
Databáze: MEDLINE
Externí odkaz: