title: A pilot study of manual chronic partial exchange transfusion in children with sickle disease.
abstract: Objective Red cell exchange transfusion is frequently used in the management of patients with sickle cell disease (SCD) either electively or chronically to maintain hemoglobin S (HbS) <30%. The purpose of this retrospective study was to evaluate the results of manual chronic partial exchange transfusion (MCPET) on level of Hb and HbS, on iron load and on the need for chelation, on risk of immunization, monitoring transfusion-transmitted viral infection, and clinical outcome. Methods We reviewed the long-term effect of MCPET in 10 children (six men and four women) with SCD and evaluated the iron balance during a median follow-up of 20 months (range: 6-36) in which 248 exchanges were performed. Results The pre-exchange median Hb value was 9.5 g/dl (range: 7.7-10.9 g/dl) and the median post-exchange value was 9.4 g/dl (range: 8.4-11.1 g/dl).The majority of patients reached an HbS of <50% with a median HbS value of 40.04% (range: 30-54). At start of the MCPET program, the median ferritin was 439 ng/ml (range: 80-1704 ng/ml). In the final evaluation, the median value of ferritin was 531 ng/ml (range: 84-3840 ng/ml). The annual calculated iron balance was 0. 28 ± 0.08 mg/kg/day. MCPET was well tolerated, and adverse effects were limited. Discussion MCPET in children with SCD is safe to prevent iron overload, and is effective and easy to use in our cohort. Conclusion Indication for chronic exchange blood transfusion is essential for patients with SCD with recurrent and frequent crises who do not respond to hydroxyurea. However, there is no consensual study for the period at which chronic transfusion can safely be stopped and further research in large population of patients with SCD will need to clarify this question.
authors.forename: Michel Ntetani
authors.initials: MN
authors.lastname: Aloni
authors.forename: Phu-Quoc
authors.initials: PQ
authors.lastname: Lê
authors.forename: Catherine
authors.initials: C
authors.lastname: Heijmans
authors.forename: Sophie
authors.initials: S
authors.lastname: Huybrechts
authors.forename: Christine
authors.initials: C
authors.lastname: Devalck
authors.forename: Nadira
authors.initials: N
authors.lastname: Azzi
authors.forename: Malou
authors.initials: M
authors.lastname: Ngalula-Mujinga
authors.forename: Alina
authors.initials: A
authors.lastname: Ferster
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D004909:Erythrocyte Indices; D005078:Exchange Transfusion, Whole Blood / Q000009:adverse effects / Q000379:methods*; D005260:Female; D005293:Ferritins / Q000097:blood; D006451:Hemoglobin, Sickle; D006454:Hemoglobins; D006801:Humans; D019190:Iron Overload / Q000188:drug therapy / Q000209:etiology; D008297:Male; D010865:Pilot Projects; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D006451:Hemoglobin, Sickle; D006454:Hemoglobins; D005293:Ferritins
keywords: Belgium; Children; Manual chronic partial exchange transfusion; Red cell exchange; Sickle cell disease; Transfusion risks