title: Fatal agranulocytosis after deferiprone therapy in a child with Diamond-Blackfan anemia.
abstract: A 10-year-old girl with steroid-resistant Diamond-Blackfan anemia (DBA) developed agranulocytosis 9 weeks after chelation with deferiprone was initiated (45 mg/kg daily, 60% of recommended dose) in addition to her ordinary deferoxamine therapy. The blood counts, checked weekly, dropped markedly between weeks 8 and 9. She rapidly developed a septicemia and was admitted with high fever (40.9 degrees C), white blood cell count 0.4 x 10(9)/L, absolute neutrophil count 0.1 x 10(9)/L and platelets 114 x 10(9)/L. She was administered broad spectrum antibiotics, G-CSF (10 microgram/kg daily) and corticosteroids but remained neutropenic and died 6 weeks after admission. Bone marrow examination day 23 revealed areas with low cellularity (around 30%), but also areas with infiltrates of T cells; granulopoiesis and erythropoiesis were scarce. We conclude that weekly neutrophil monitoring is not sufficient to avoid fatal agranulocytosis. We suggest that deferiprone not be prescribed to DBA patients unless the clinical indications are particularly strong, and that the risk of agranulocytosis in thalassemia patients be carefully considered.
authors.affiliation: Childhood Cancer Research Unit, Department of Woman and Child Health, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden. Jan-Inge.Henter@ki.se
authors.forename: Jan-Inge
authors.initials: JI
authors.lastname: Henter
authors.forename: Jonas
authors.initials: J
authors.lastname: Karlén
mesh_terms: D000380:Agranulocytosis / Q000139:chemically induced*; D029503:Anemia, Diamond-Blackfan / Q000150:complications* / Q000188:drug therapy*; D001772:Blood Cell Count; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D000075202:Contraindications; D000077543:Deferiprone; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D011728:Pyridones / Q000009:adverse effects* / Q000627:therapeutic use; D013789:Thalassemia / Q000150:complications
chemical_list: D011728:Pyridones; D000077543:Deferiprone