Iron Overload in Myelodysplastic Syndromes: Pathophysiology, Consequences, Diagnosis, and Treatment.

摘要: Myelodysplastic syndromes (MDS) are a heterogeneous group of hematologic neoplasms varying in severity affecting one or more lines hematopoiesis. Ineffective erythropoiesis results dysregulation iron metabolism. Most MDS patients have anemia, and some require regular red blood cell transfusions. These transfusions, addition to factors the disease itself, can result overload (IO). Retrospective analyses suggest that with IO reduced overall survival poorer outcomes following allogeneic stem transplant vs. those without IO. Iron chelation therapy (ICT; deferoxamine, deferasirox, deferiprone) has been used alleviate other transfusion-dependent conditions (e.g., thalassemia), but its role not firmly established. A growing body evidence suggests ICT is an effective means for reducing transfusional may significantly improve such as survival. The orally administered chelator deferasirox widely studied MDS, available studies shown it be generally well tolerated this population. pathophysiology clinical consequences current methods diagnosing treating these patients, discussed.