title: Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention.
abstract: Chronic transfusion reduces the risk of recurrent stroke in children with sickle cell anemia (SCA) but leads to iron loading. Management of transfusional iron overload in SCA has been reported as suboptimal [1], but studies characterizing monitoring and treatment practices for iron overload in children with SCA, particularly in recent years with the expansion of chelator options, are lacking. We investigated the degree of iron loading and treatment practices of 161 children with SCA receiving transfusions for a history of stroke who participated in the Stroke with Transfusions Changing to Hydroxyurea (SWiTCH) trial. Data obtained during screening, including past and entry liver iron concentration (LIC) measurements, ferritin values, and chelation were analyzed. The mean age at enrollment was 12.9 ± 4 years and the mean duration of transfusion was 7 ± 3.8 years. Baseline LIC (median 12.94 mg/g dw) and serum ferritin (median 3,164 ng/mL) were elevated. Chelation therapy was initiated after a mean of 2.6 years of transfusions. At study entry, 137 were receiving chelation, most of whom (90%) were receiving deferasirox. This study underscores the need for better monitoring of iron burden with timely treatment adjustments in chronically transfused children with SCA.
authors: Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. kwiatkowski@email.chop.edu
authors: Janet L
authors: JL
authors: Kwiatkowski
authors: Alan R
authors: AR
authors: Cohen
authors: Julian
authors: J
authors: Garro
authors: Ofelia
authors: O
authors: Alvarez
authors: Ramamorrthy
authors: R
authors: Nagasubramanian
authors: Sharada
authors: S
authors: Sarnaik
authors: Alexis
authors: A
authors: Thompson
authors: Gerald M
authors: GM
authors: Woods
authors: William
authors: W
authors: Schultz
authors: Nicole
authors: N
authors: Mortier
authors: Peter
authors: P
authors: Lane
authors: Brigitta
authors: B
authors: Mueller
authors: Nancy
authors: N
authors: Yovetich
authors: Russell E
authors: RE
authors: Ware
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D001565:Benzoates / Q000494:pharmacology / Q000627:therapeutic use*; D015913:Chelation Therapy; D002648:Child; D000077588:Deferasirox; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000494:pharmacology / Q000627:therapeutic use*; D019190:Iron Overload / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D008099:Liver / Q000378:metabolism; D008297:Male; D055502:Secondary Prevention; D020521:Stroke / Q000517:prevention & control*; D065227:Transfusion Reaction; D014230:Triazoles / Q000494:pharmacology / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D001565:Benzoates; D007502:Iron Chelating Agents; D014230:Triazoles; D005293:Ferritins; D007501:Iron; D000077588:Deferasirox