Tolerance induction to deferasirox in a child with transfusion-dependent beta thalassemia

Beta thalassemias are autosomal recessive hemoglobin disorders related to a defect in the beta-globin chain production. Most of the major forms of beta-thalassemia are transfusion dependent leading to iron overload. Today, three iron chelators are available in France. We report the case of a patient suffering from beta(+) major transfusion-dependent thalassemia who presented with severe skin reactions to deferoxamine and deferasirox as well as with agranulocytosis after deferiprone administration. The patient benefited from successful tolerance induction to deferasirox. With the increasing number of children suffering from iron overload, we believe that our protocol can be useful to pediatric hematology teams confronted with multiple iron chelator reactions. (C) 2020 Published by Elsevier Masson SAS on behalf of French Society of Pediatrics.

Automated summary

Beta thalassemias are hemoglobin disorders related to a defect in beta-globin chain production, often leading to transfusion dependence and iron overload. A case study of a patient with beta(+) major transfusion-dependent thalassemia showed successful tolerance induction to deferasirox after severe reactions to deferoxamine, deferiprone, and agranulocytosis. This protocol may be useful for pediatric hematology teams dealing with multiple iron chelator reactions in children with iron overload.