Tolerance induction to deferasirox in a child with transfusion-dependent beta thalassemia. 2021

Morgane Pondrom, and Fabrice Monpoux, and Fanny Rocher, and Nadia Gastaut, and Carole Bailly-Piccini, and Maryline Poirée
Unité d'Hémato-Oncologie Infantile. Hôpital de l'Archet 2, 151, route de Saint Antoine de Ginestière, 06202 Nice Cedex 3, France.

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 β+ 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.

UI MeSH Term Description Entries
D007108 Immune Tolerance The specific failure of a normally responsive individual to make an immune response to a known antigen. It results from previous contact with the antigen by an immunologically immature individual (fetus or neonate) or by an adult exposed to extreme high-dose or low-dose antigen, or by exposure to radiation, antimetabolites, antilymphocytic serum, etc. Immunosuppression (Physiology),Immunosuppressions (Physiology),Tolerance, Immune
D007223 Infant A child between 1 and 23 months of age. Infants
D007502 Iron Chelating Agents Organic chemicals that form two or more coordination links with an iron ion. Once coordination has occurred, the complex formed is called a chelate. The iron-binding porphyrin group of hemoglobin is an example of a metal chelate found in biological systems. Iron Chelates,Agents, Iron Chelating,Chelates, Iron,Chelating Agents, Iron
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003875 Drug Eruptions Adverse cutaneous reactions caused by ingestion, parenteral use, or local application of a drug. These may assume various morphologic patterns and produce various types of lesions. Dermatitis Medicamentosa,Dermatitis, Adverse Drug Reaction,Maculopapular Drug Eruption,Maculopapular Exanthem,Morbilliform Drug Reaction,Morbilliform Exanthem,Drug Eruption,Drug Eruption, Maculopapular,Drug Eruptions, Maculopapular,Drug Reaction, Morbilliform,Drug Reactions, Morbilliform,Eruption, Drug,Eruption, Maculopapular Drug,Eruptions, Drug,Eruptions, Maculopapular Drug,Exanthem, Maculopapular,Exanthem, Morbilliform,Exanthems, Maculopapular,Exanthems, Morbilliform,Maculopapular Drug Eruptions,Maculopapular Exanthems,Morbilliform Drug Reactions,Morbilliform Exanthems,Reaction, Morbilliform Drug,Reactions, Morbilliform Drug
D003888 Desensitization, Immunologic Immunosuppression by the administration of increasing doses of antigen. Though the exact mechanism is not clear, the therapy results in an increase in serum levels of allergen-specific IMMUNOGLOBULIN G, suppression of specific IgE, and an increase in suppressor T-cell activity. Allergen Immunotherapy,Allergy Shots,Hyposensitization Therapy,Immunotherapy, Allergen,Venom Immunotherapy,Immunologic Desensitization,Therapy, Hyposensitization,Allergen Immunotherapies,Allergy Shot,Desensitizations, Immunologic,Hyposensitization Therapies,Immunologic Desensitizations,Immunotherapy, Venom,Shot, Allergy,Venom Immunotherapies
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000077588 Deferasirox A triazole and benzoate derivative that acts as a selective iron chelator. It is used in the management of chronic IRON OVERLOAD due to blood transfusion or non-transfusion dependent THALASSEMIA. 4-(3,5-Bis-(2-hydroxyphenyl)-(1,2,4)-triazol-1-yl)benzoic acid,Exjade,ICL 670,ICL 670A,ICL-670,ICL-670A,ICL670,ICL670A
D017086 beta-Thalassemia A disorder characterized by reduced synthesis of the beta chains of hemoglobin. There is retardation of hemoglobin A synthesis in the heterozygous form (thalassemia minor), which is asymptomatic, while in the homozygous form (thalassemia major, Cooley's anemia, Mediterranean anemia, erythroblastic anemia), which can result in severe complications and even death, hemoglobin A synthesis is absent. Anemia, Cooley's,Anemia, Erythroblastic,Anemia, Mediterranean,Hemoglobin F Disease,Thalassemia Major,Thalassemia Minor,Erythroblastic Anemia,Mediterranean Anemia,Microcytemia, beta Type,Thalassemia Intermedia,Thalassemia Major (beta-Thalassemia Major),Thalassemia Minor (beta-Thalassemia Minor),Thalassemia, beta Type,beta Thalassemia,Anemia, Cooley,Anemia, Cooleys,Anemias, Erythroblastic,Anemias, Mediterranean,Cooley's Anemia,Disease, Hemoglobin F,Intermedia, Thalassemia,Intermedias, Thalassemia,Major, Thalassemia (beta-Thalassemia Major),Majors, Thalassemia (beta-Thalassemia Major),Mediterranean Anemias,Microcytemias, beta Type,Minor, Thalassemia (beta-Thalassemia Minor),Minors, Thalassemia (beta-Thalassemia Minor),Thalassemia Intermedias,Thalassemia Major (beta Thalassemia Major),Thalassemia Majors (beta-Thalassemia Major),Thalassemia Minor (beta Thalassemia Minor),Thalassemia Minors (beta-Thalassemia Minor),Thalassemia, beta,Thalassemias, beta,Thalassemias, beta Type,Type Microcytemia, beta,Type Microcytemias, beta,Type Thalassemia, beta,Type Thalassemias, beta,beta Thalassemias,beta Type Microcytemia,beta Type Microcytemias,beta Type Thalassemia,beta Type Thalassemias

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