Iron status in a cohort of children with sickle cell disease on chronic red cell exchange

Anthea L. Greenway, Anna Duncan, Brendan Cusack, Zoe Millard, Lucy Dove, Amanda Walker, David Kieran Metz, Jacqueline Flemming

Research output: Contribution to journalMeeting Abstractpeer-review

Abstract

Purpose: Chronic Red cell exchange (RCE) may be used to manage/prevent complications of Sickle cell Disease (SCD) including stroke or recurrent vaso-occlusive crises. RCE has been described as effective therapy to manage transfusion associated iron overload in patients with SCD who require ongoing transfusion support. Significant variation in iron status has been noted in the RCH cohort of children on regular RCE with sickle cell disease, from transfusion associated siderosis to iron deficiency.An audit was undertaken to describe the iron status, and requirement for iron chelation or iron supplementation for a cohort of children with SCD requiring RCE at The Royal Children’s Hospital, Melbourne.
Methods: A Retrospective audit of iron status (ferritin and Ferriscan) of children managed with chronic RCE for management of SCD was undertaken for the last 6 years. Medical records were reviewed to consider effects of regular RCE on iron status including : ferritin, change in serum ferritin from prior to commencing RCE to current status, requirement for iron chelation or treatment of iron deficiency, and assessment of organ involvement (Ferriscan) for children under 18 managed on the RCE program at RCH.
Results: Clinical data related to 264 RCE procedures, performed in 9 patients will be presented. Ferritin was reduced over the audit period in 5 patients, with an additional patient noted to have stable levels on RCE without iron chelation. Iron deficiency (ferritin < 20 ug/L) developed in two patients, both adolescent males (including one patient whom had previously required oral iron chelation for 5 years prior to starting RCE). Both patients were treated with oral iron supplements. Reduction in hepatic iron overload was observed in 3 patients (improved Liver Iron Content on Ferriscan), with maintenance of normal hepatic iron despite ongoing RCE in a remaining 2 patients. 4 patients were able to cease oral iron chelation whilst continuing RCE, 2 patients have subsequently recommenced iron chelation. No patient had evidence of cardiac iron deposition.
Conclusion: Chronic RCE was observed to reduce iron overload or prevent worsening transfusion associated iron overload (combined with iron chelation), in our cohort of children with SCD. Iron deficiency was also observed in a small number of patients. Oral iron supplementation was effective to maintain serum ferritin with good symptomatic effect. Parenteral iron therapy may also be useful (however is more expensive, with some associated risks). Clinicians should be aware of the risk of developing iron deficiency. Underlying factors which influence development of iron overload or iron deficiency in association with regular RCE for children with Sickle cell disease are yet to be established. Further studies are needed to compare oral versus parenteral iron replacement in this cohort and to determine the incidence of RCE associated iron deficiency.
Original languageEnglish
Article number29 P-19
Pages (from-to)251-252
Number of pages2
JournalJournal of Clinical Apheresis
Volume36
Issue number2
DOIs
Publication statusPublished - Apr 2021
Externally publishedYes
EventAmerican Society for Apheresis Annual Meeting 2021 - virtual meeting
Duration: 12 May 202115 May 2021
https://onlinelibrary.wiley.com/toc/10981101/2021/36/2

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