Iron Overload in Myelodysplastic Syndromes: Diagnosis and Management

Alan F. List
2010 Cancer Control: Journal of the Moffitt Cancer Cente  
all. 1.2 Approximately two-thirds of patients have lowerrisk disease as defined by the International Prognostic Scoring System (IPSS), 3,4 which includes the categories of low-risk and intermediate-1 (Int-1)-risk disease. These categories tend to have an indolent clinical course and expectation for a prolonged survival. Overall, it is estimated that 39% of patients with low-and Int-1-risk MDS will require regular red blood cell (RBC) transfusions for management of chronic anemia symptoms and
more » ... mia symptoms and thus are at risk for complications arising from iron overload. 5 RBC transfusion dependence was only recently recognized to have prognostic implications for disease behavior separate from the effect of iron loading. In a retrospective analysis by investigators at the University of Pavia, 6 RBC transfusion dependence was associated with a 36% reduction in survival for every 500 μg/L increase in serum ferritin above 1,000 μg/L. Similarly, baseline transfusion dependence and iron overload were independent prognostic Iron Overload in Myelodysplastic Syndromes: Diagnosis and Management Alan F. List, MD Myelodysplastic syndrome (MDS) is composed of a diverse spectrum of hematopoietic stem cell malignancies characterized by ineffective blood cell production. Many MDS patients are dependent on red blood cell (RBC) transfusions for symptomatic management of refractory anemia. Iron overload ensues when the iron acquired from transfused RBCs exceeds body storage capacity, thereby raising the risk for end organ damage. This is of greatest concern in patients with lower-risk MDS whose expected survival is measured in years. Transfusion dependence is associated with shorter survival and an increased risk for progression to acute myeloid leukemia (AML) in transfusion-dependent patients. Application of recent advances in the treatment of MDS can reduce or eliminate the need for transfusions, thus minimizing the risk of iron overload. Case control studies, prospective surveys, and phase II studies indicate that iron chelation therapy reduces iron load as measured by changes in serum ferritin and may prolong overall survival. Iron chelation strategies include oral agents such as deferasirox (Exjade ® , Novartis Pharmaceuticals Corp, East Hanover, NJ), deferiprone (Ferriprox ® , Apotex Europe BV, Leiden, the Netherlands) and, for those patients who are intolerant of or for whom oral therapy is ineffective, parenteral administration of deferoxamine (Desferal ® , Novartis). This review presents the data related to iron overload in MDS, including its prevalence, diagnosis, clinical impact, and management. Iron overload is a deleterious treatment-related complication for some MDS patients.
doi:10.1177/107327481001701s01 pmid:20125080 fatcat:owrfkvvz65epbetihj36mhx574