title: Fanconi Syndrome Secondary to Deferasirox in Diamond-Blackfan Anemia: Case Series and Recommendations for Early Diagnosis.
abstract: Deferasirox is an oral iron chelator used to treat patients with transfusion-related iron overload. We report, from two institutions, two children with Diamond-Blackfan anemia who developed Fanconi syndrome secondary to deferasirox administration, along with a review of the literature. The current recommendation for the laboratory monitoring of patients receiving deferasirox does not include serum electrolytes or urine analysis. Thus, despite routine clinic visits and bloodwork, these two patients presented with life-threatening electrolyte abnormalities requiring hospitalization. Hence, we propose the inclusion of serum electrolytes and urine analysis as part of routine monitoring to facilitate the early diagnosis of Fanconi syndrome in the context of high doses of deferasirox therapy.
authors.affiliation: Division of Pediatric Hematology/Oncology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Koyelle
authors.initials: K
authors.lastname: Papneja
authors.affiliation: Division of Pediatric Hematology/Oncology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Mihir D
authors.initials: MD
authors.lastname: Bhatt
authors.affiliation: Division of Pediatric Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Melanie
authors.initials: M
authors.lastname: Kirby-Allen
authors.affiliation: Division of Nephrology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Steven
authors.initials: S
authors.lastname: Arora
authors.affiliation: Division of Pediatric Hematology/Oncology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.
authors.forename: John T
authors.initials: JT
authors.lastname: Wiernikowski
authors.affiliation: Division of Pediatric Hematology/Oncology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Uma H
authors.initials: UH
authors.lastname: Athale
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D029503:Anemia, Diamond-Blackfan / Q000188:drug therapy*; D001565:Benzoates / Q000627:therapeutic use*; D001803:Blood Transfusion / Q000379:methods; D002648:Child; D000077588:Deferasirox; D004573:Electrolytes / Q000097:blood / Q000652:urine; D005198:Fanconi Syndrome / Q000139:chemically induced*; D005260:Female; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D019190:Iron Overload / Q000188:drug therapy* / Q000517:prevention & control; D008297:Male; D065227:Transfusion Reaction; D014230:Triazoles / Q000627:therapeutic use*
chemical_list: D001565:Benzoates; D004573:Electrolytes; D007502:Iron Chelating Agents; D014230:Triazoles; D000077588:Deferasirox
keywords: Diamond-Blackfan anemia; Fanconi syndrome; chronic transfusion therapy; deferasirox; iron chelation