Znaczenie terapii chelatujacej u pacjentów z przeładowaniem zelaza w wyniku czestych transfuzji krwi

Abstract

The most common cause of iron overload (IO) are red blood cells transfusions, in less extend also increase of iron absorption from the gut due to ineffective erythropoiesis. IO leads to liver fibrosis/cirrhosis, cardiac dysfunction, diabetes and other endocrinopathies, changes in immunity worsening of survival in patients undergoing allogenic hematopoietic stem cell transplantation (allo-HSCT). To prevent and delay complications associated with iron deposition in organs, iron chelation therapy with deferoxamine, deferiprone, deferasirox in monotherapy or in combination is recommended. Chelation therapy should be considered in transfussion-dependent patients diagnosed with inherited anemias (sickle cell anemia, thalassemias), low risk myelodysplastic syndromes, primary idiopatic myelofibrosis, with ferritin concentration higher than 1000 ng/mLor with iron depositions (confirmed in liver biopsy or nuclear magnetic resonance). Patients who have undergone allo-HSCT with persistent IO may also be candidates for treatment with iron chelators. We have more consistent data and evidences that chelating therapy improves the function of damaged organs, improves hematological response, and prolongs the survival of patients.