title: Transfusion management of sickle cell patients during bone marrow transplantation with matched sibling donor.
abstract: BACKGROUND
  Sickle cell disease (SCD) patients have unique transfusion considerations during bone marrow transplantation (BMT), including prophylaxis against stroke and alloimmunization. Characterization of transfusion requirements is important for blood bank and clinician patient management.
  
  
  METHODS
  A retrospective analysis of red blood cell (RBC) and platelet (PLT) transfusion of SCD patients during myeloablative matched sibling donor (MSD) BMT at one institution from 1993 to 2007 was performed. Patient characteristics (RBC blood group antibodies, ABO-incompatible donor, BMT-related morbidity) and transfusion practices (RBC phenotype matching, transfusion threshold, and blood age) were assessed for effect on total RBC transfusion volumes.
  
  
  RESULTS
  Twenty-seven patients received MSD BMT with 96% survival and 0% rejection. Six alloimmunized patients received RBCs with extended phenotype matching (C, c, E, e, K, Fy(a), Jk(b)), 14 nonalloimmunized received limited matching (C, c, E, e, K), and 7 did not have phenotype matching. Among 26 survivors, a median seven RBC transfusions (range, 3-15) and 13.5 PLT transfusions (range, 4-48) per patient were administered, equivalent to 64 mL/kg RBCs (range, 22-122 mL/kg) and 106 mL/kg PLTs (range, 26-343 mL/kg). BMT-related morbidity predicted increased RBC transfusions (p = 0.006). Venoocclusive disease was associated with greater RBC (p = 0.016) and PLT transfusion volumes (p = 0.016). Greater phenotype matching was associated with decreased RBC transfusions (p = 0.0247).
  
  
  CONCLUSIONS
  SCD patients have high transfusion support during MSD BMT. Communication of BMT complications to the blood bank is essential for transfusion inventory management. Phenotype matching decreased RBC transfusions in this cohort and warrants further investigation in SCD transfusion therapy.
authors.affiliation: Aflac Cancer Center and Blood Disorders Service, Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, USA. marianne.mcpherson@choa.org
authors.forename: Marianne E
authors.initials: ME
authors.lastname: McPherson
authors.forename: Alan R
authors.initials: AR
authors.lastname: Anderson
authors.forename: Ann E
authors.initials: AE
authors.lastname: Haight
authors.forename: Paula
authors.initials: P
authors.lastname: Jessup
authors.forename: Marta-Inés
authors.initials: MI
authors.lastname: Castillejo
authors.forename: Christopher D
authors.initials: CD
authors.lastname: Hillyer
authors.forename: Cassandra D
authors.initials: CD
authors.lastname: Josephson
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000628:therapy*; D016026:Bone Marrow Transplantation / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D017707:Erythrocyte Transfusion; D005260:Female; D006801:Humans; D008297:Male; D010641:Phenotype; D017713:Platelet Transfusion; D012189:Retrospective Studies; D035781:Siblings; D014019:Tissue Donors