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Use of Hematopoietic Stem Cell Transplantation to Assess the Origin of Myelodysplastic Syndrome
Published on: October 3, 2018
The deleterious effects of iron overload in patients with myelodysplastic syndromes
1Service d'Hématologie, Hôopital Cochin, AP-HP, Paris, France. francois.dreyfus@cch.aphp.fr
Many patients with myelodysplastic syndromes (MDS) have severe anaemia. However, regular blood transfusions, which are widely used to maintain quality of life and prevent anaemia-related morbidity and mortality, have a negative impact on survival as a result of iron overload. Retrospective surveys have shown an association of transfusion dependence with hepatic, pituitary, and pancreatic dysfunction, cardiac failure, and cardiac death. Survival is significantly decreased in transfusion-dependent patients, and the main cause of non-leukaemic death is cardiac failure. However, iron chelation therapy reduces serum ferritin levels and is associated with significantly improved survival in patients with MDS. Current guidelines recommend starting iron chelation therapy after 25-50 units of blood have been transfused, or when serum ferritin levels rise above 1,000-2,000 microg/L. The patients who are most likely to benefit from iron chelation therapy are those who have low-risk disease (International Prognostic Scoring System low or intermediate-1 risk) with a life expectancy of more than 1 year. More specific studies in patients with MDS are needed to evaluate the impact of iron chelation therapy on morbidity and mortality, and provide a stronger evidence base for treatment guidelines.
Many patients with myelodysplastic syndromes (MDS) have severe anaemia. However, regular blood transfusions, which are widely used to maintain quality of life and prevent anaemia-related morbidity and mortality, have a negative impact on survival as a result of iron overload. Retrospective surveys have shown an association of transfusion dependence with hepatic, pituitary, and pancreatic dysfunction, cardiac failure, and cardiac death. Survival is significantly decreased in transfusion-dependent patients, and the main cause of non-leukaemic death is cardiac failure. However, iron chelation therapy reduces serum ferritin levels and is associated with significantly improved survival in patients with MDS. Current guidelines recommend starting iron chelation therapy after 25-50 units of blood have been transfused, or when serum ferritin levels rise above 1,000-2,000 microg/L. The patients who are most likely to benefit from iron chelation therapy are those who have low-risk disease (International Prognostic Scoring System low or intermediate-1 risk) with a life expectancy of more than 1 year. More specific studies in patients with MDS are needed to evaluate the impact of iron chelation therapy on morbidity and mortality, and provide a stronger evidence base for treatment guidelines.
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