Pondrom Morgane, Monpoux Fabrice, Rocher Fanny, Gastaut Nadia, Bailly-Piccini Carole, Poirée Maryline
Unité d'Hémato-Oncologie Infantile. Hôpital de l'Archet 2, 151, route de Saint Antoine de Ginestière, 06202 Nice Cedex 3, France.
Unité d'Hémato-Oncologie Infantile. Hôpital de l'Archet 2, 151, route de Saint Antoine de Ginestière, 06202 Nice Cedex 3, France.
Arch Pediatr. 2021 Jan;28(1):101-103. doi: 10.1016/j.arcped.2020.10.010. Epub 2020 Nov 26.
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.
β地中海贫血是一种常染色体隐性血红蛋白疾病，与β珠蛋白链生成缺陷有关。大多数主要形式的β地中海贫血依赖输血，会导致铁过载。如今，法国有三种铁螯合剂可供使用。我们报告了一例患有重度输血依赖型β地中海贫血的患者，该患者对去铁胺和地拉罗司出现了严重的皮肤反应，并且在服用去铁酮后出现了粒细胞缺乏症。该患者成功诱导了对地拉罗司的耐受性。随着患铁过载的儿童数量不断增加，我们认为我们的方案对面临多种铁螯合剂反应的儿科血液学团队可能有用。
