title: The use of erythropoietin-stimulating agents versus supportive care in newborns with hereditary spherocytosis: a single centre's experience.
abstract: Hereditary Spherocytosis (HS) is a common haemolytic anaemia in which 75% of cases are autosomal dominant. As most newborns with HS have a family history of disease, haematologists often see these infants before their physiologic haemoglobin nadir, which is exaggerated in comparison with healthy infants. The objective of this study was to evaluate the frequency of implementation and cost of erythropoietin-stimulating agents (EPO) versus transfusion in infants with HS at a single paediatric programme. In the last decade, only 15% of infants with HS at our centre have been treated with EPO, which costs twice that of a single transfusion and EPO treated infants did not always avoid transfusion. Infrequent prescription of EPO therapy to infants with HS at our centre may be related to the incomplete data supporting its use.
authors.affiliation: Division of Hematology/Oncology, Boston Children's Hospital, Boston, MA, USA.
authors.forename: Jacqueline F
authors.initials: JF
authors.lastname: Morrison
authors.forename: Ellis J
authors.initials: EJ
authors.lastname: Neufeld
authors.forename: Rachael F
authors.initials: RF
authors.lastname: Grace
mesh_terms: D001724:Birth Weight; D017707:Erythrocyte Transfusion / Q000191:economics*; D004921:Erythropoietin / Q000191:economics / Q000627:therapeutic use*; D005260:Female; D005865:Gestational Age; D006397:Hematinics / Q000191:economics / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012720:Severity of Illness Index; D013103:Spherocytosis, Hereditary / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D006397:Hematinics; D004921:Erythropoietin
keywords: erythropoietin; hereditary spherocytosis; paediatric