title: Prospective RBC phenotype matching in a stroke-prevention trial in sickle cell anemia: a multicenter transfusion trial.
abstract: BACKGROUND
  Most sickle cell anemia patients undergo transfusion therapy to prevent complications. The Stroke Prevention Trial in Sickle Cell Anemia showed that transfusion therapy is effective in the primary prevention of stroke. Despite its efficacy, transfusion therapy is limited by alloimmunization. The purpose of this study was to determine if a multicenter trial could implement a transfusion program utilizing phenotypically matched blood to reduce alloimmunization.
  
  
  METHODS
  One hundred thirty children underwent RBC phenotyping and antibody screening with review of blood bank records. The protocol required use of WBC-reduced RBCs, which were matched for E, C, and Kell. Monthly alloantibody testing and review of transfusion forms were performed to determine compliance and the occurrence of any adverse events.
  
  
  RESULTS
  Patient RBCs expressed a low frequency of Kell (2%), E (20%), and C (25%) antigens. Sixty-one patients received 1830 units. Ninety-seven percent of all units were WBC reduced. Only 29 units were inadvertently not matched for E, C, and Kell. Five patients (8%) developed a clinically significant alloantibody. Four developed a single antibody to E or Kell. Three patients (5%) developed a warm autoantibody. There were 11 transfusion reactions and 8 transfusion-associated events. Transfusion reactions included 6 febrile reactions (0.33%/unit), 3 allergic (0.16%/unit), and 2 hemolytic (0.11%/unit). Associated events included 4 episodes of hypertension (0.22%/unit), 3 crises (0.16%/unit), and 1 transient ischemic attack (0.05%/unit).
  
  
  CONCLUSIONS
  This is the first multicenter study to show that extended RBC phenotyping can be implemented nationwide. Compared to studies, the alloimmunization rate dropped from 3 percent to 0.5 percent per unit, and hemolytic transfusion reactions dropped by 90 percent. It is recommended that all transfused sickle cell anemia patients be antigen matched for E, C, and Kell. Patients should be closely monitored during transfusions to avoid preventable risks.
authors.affiliation: Department of Hematology/Oncology, Children's Hospital Oakland, Oakland, California 94609, USA. evichinsky@mail.cho.org
authors.forename: E P
authors.initials: EP
authors.lastname: Vichinsky
authors.forename: N L
authors.initials: NL
authors.lastname: Luban
authors.forename: E
authors.initials: E
authors.lastname: Wright
authors.forename: N
authors.initials: N
authors.lastname: Olivieri
authors.forename: C
authors.initials: C
authors.lastname: Driscoll
authors.forename: C H
authors.initials: CH
authors.lastname: Pegelow
authors.forename: R J
authors.initials: RJ
authors.lastname: Adams
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000276:immunology / Q000628:therapy*; D001323:Autoantibodies / Q000032:analysis; D001788:Blood Grouping and Crossmatching; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D004912:Erythrocytes / Q000502:physiology*; D005260:Female; D006801:Humans; D007518:Isoantibodies / Q000032:analysis; D008297:Male; D010641:Phenotype; D011446:Prospective Studies; D020521:Stroke / Q000517:prevention & control*; D065227:Transfusion Reaction
chemical_list: D001323:Autoantibodies; D007518:Isoantibodies