title: Comparison of automated erythrocytapheresis versus manual exchange transfusion to treat cerebral macrovasculopathy in sickle cell anemia.
abstract: Chronic exchange transfusion is effective for primary and secondary prevention of stroke in children with sickle cell anemia (SCA). Erythrocytapheresis is recognized to be the most efficient approach; however, it is not widely implemented and is not suitable for all patients. The aim of our study was to compare automated exchange transfusion (AET) with our manual method of exchange transfusion and, in particular, to evaluate the efficacy, safety, and cost of our manual method.
  
  
  
  Thirty-nine SCA children with stroke and/or abnormal findings on transcranial Doppler were included in the study. We retrospectively analyzed 1353 exchange sessions, including 333 sessions of AET and 1020 sessions of manual exchange transfusion (MET).
  
  
  
  Both methods were well tolerated. The median decrease in hemoglobin (Hb)S per session was 21.5% with AET and 18.8% with our manual method (p < 0.0001) with no major increase in red blood cell consumption. Iron overload was well controlled, even with the manual method, with a median (interquartile range) ferritin level of 312 (152-994) µg/L after 24 months of transfusions. The main differences in annual cost relate to equipment costs, which were 74 times higher with the automated method.
  
  
  
  Our study shows that continuous MET has comparable efficacy to the automated method in terms of stroke prevention, decrease in HbS, and iron overload prevention. It is feasible in all hospital settings and is often combined with AET successively over time.
authors.affiliation: Pediatric Unit, AP-HP, Robert Debré Hospital, Paris.|Université Paris 7-Diderot, Paris.
authors.forename: Bérengère
authors.initials: B
authors.lastname: Koehl
authors.affiliation: Université Paris 7-Diderot, Paris.|Clinic Epidemiology Unit, AP-HP, Robert Debré Hospital, Paris.|INSERM UMR 1123, Paris, France.
authors.forename: Julie
authors.initials: J
authors.lastname: Sommet
authors.affiliation: Hematology Unit, AP-HP, Robert Debré Hospital, Paris.|Reference Centre of Sickle Cell Disease, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Laurent
authors.initials: L
authors.lastname: Holvoet
authors.affiliation: Clinic Epidemiology Unit, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Hendy
authors.initials: H
authors.lastname: Abdoul
authors.affiliation: Clinic Epidemiology Unit, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Priscilla
authors.initials: P
authors.lastname: Boizeau
authors.affiliation: Hematology Unit, AP-HP, Robert Debré Hospital, Paris.|Reference Centre of Sickle Cell Disease, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Ghislaine
authors.initials: G
authors.lastname: Ithier
authors.affiliation: Hematology Unit, AP-HP, Robert Debré Hospital, Paris.|Reference Centre of Sickle Cell Disease, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Florence
authors.initials: F
authors.lastname: Missud
authors.affiliation: Biochemistry and Molecular Genetics, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Couque
authors.affiliation: Radiology Unit, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Suzanne
authors.initials: S
authors.lastname: Verlhac
authors.affiliation: Etablissement Français du Sang, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Pauline
authors.initials: P
authors.lastname: Voultoury
authors.affiliation: Etablissement Français du Sang, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Fatiha
authors.initials: F
authors.lastname: Sellami
authors.affiliation: Université Paris 7-Diderot, Paris.|Hematology Unit, AP-HP, Robert Debré Hospital, Paris.
authors.forename: André
authors.initials: A
authors.lastname: Baruchel
authors.affiliation: INSERM UMR 1123, Paris, France.|Hematology Unit, AP-HP, Robert Debré Hospital, Paris.|Reference Centre of Sickle Cell Disease, AP-HP, Robert Debré Hospital, Paris.
authors.forename: Malika
authors.initials: M
authors.lastname: Benkerrou
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000628:therapy; D001331:Automation; D002561:Cerebrovascular Disorders / Q000209:etiology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D016238:Cytapheresis / Q000191:economics / Q000295:instrumentation* / Q000379:methods; D004912:Erythrocytes; D005078:Exchange Transfusion, Whole Blood / Q000191:economics / Q000379:methods*; D005260:Female; D005293:Ferritins / Q000097:blood; D006451:Hemoglobin, Sickle / Q000032:analysis; D006801:Humans; D019190:Iron Overload; D008297:Male; D012189:Retrospective Studies; D020521:Stroke / Q000209:etiology / Q000628:therapy
chemical_list: D006451:Hemoglobin, Sickle; D005293:Ferritins