Veerle Compernolle1, Stella T Chou2, Susano Tanael3, William Savage4, Jo Howard5, Cassandra D Josephson6, Isaac Odame7, Christopher Hogan8, Gregory Denomme9, Nadine Shehata3,10. 1. Belgian Red Cross-Flanders, Belgium. 2. The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania. 3. Center for Innovation, Canadian Blood Services, Toronto, Canada. 4. Blood Bank, Division of Transfusion Medicine, Brigham and Women's Hospital, Boston, Massachusetts. 5. Guy's and St. Thomas' NHS Foundation Trust, London, United Kingdom. 6. Departments of Pathology and Pediatrics, Emory University School of Medicine, and Blood, Tissue and Apheresis Services, Children's Healthcare of Atlanta, Atlanta, Georgia. 7. Departments of Paediatrics and Medicine, University of Toronto, Division of Paediatric and Adult Haematology, Hospital for Sick Children, University of Toronto, Toronto, Canada. 8. Pathology Services, Australian Red Cross Blood Services; Austin Hospital; and The Royal Melbourne Hospital, Melbourne, Australia. 9. Diagnostic Laboratories and Blood Research Institute, BloodCenter of Wisconsin, Milwaukee, Wisconsin. 10. Department of Medicine, University of Toronto, Division of Hematology, Mount Sinai Hospital, Toronto, Canada.
Abstract
BACKGROUND: Red blood cell (RBC) transfusions remain essential in the treatment of patients with sickle cell disease (SCD) and β-thalassemia. Alloimmunization, a well-documented complication of transfusion, increases the risk of delayed hemolytic transfusion reactions, complicates crossmatching and identifying compatible units, and delays provision of transfusions. Guidance is required to optimize the RBC product administered to these patients. STUDY DESIGN AND METHODS: An international, multidisciplinary team conducted a systematic review and developed, following the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology, recommendations to assist treating physicians and transfusion specialists in their decision to select RBCs for these patients. RESULTS: Eighteen studies (17 clinical studies and one cost-effectiveness study) were included in the systematic review. The overall quality of the studies was very low. In total, 3696 patients were included: 1680 with β-thalassemia and 2016 with SCD. CONCLUSION: The panel recommends that ABO D CcEe K-matched RBCs are selected for individuals with SCD and β-thalassemia, even in the absence of alloantibodies, to reduce the risk of alloimmunization. In patients with SCD and β-thalassemia who have developed clinically significant alloantibodies, selection of RBCs antigen negative to the alloantibody is recommended, if feasible. In these patients, selection of more extended phenotype-matched RBCs will likely reduce the risk of further alloimmunization. However, given the limited availability of extended phenotype-matched units, attention should be given to ensure that a delay in transfusion does not adversely affect patient care.
BACKGROUND: Red blood cell (RBC) transfusions remain essential in the treatment of patients with sickle cell disease (SCD) and β-thalassemia. Alloimmunization, a well-documented complication of transfusion, increases the risk of delayed hemolytic transfusion reactions, complicates crossmatching and identifying compatible units, and delays provision of transfusions. Guidance is required to optimize the RBC product administered to these patients. STUDY DESIGN AND METHODS: An international, multidisciplinary team conducted a systematic review and developed, following the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology, recommendations to assist treating physicians and transfusion specialists in their decision to select RBCs for these patients. RESULTS: Eighteen studies (17 clinical studies and one cost-effectiveness study) were included in the systematic review. The overall quality of the studies was very low. In total, 3696 patients were included: 1680 with β-thalassemia and 2016 with SCD. CONCLUSION: The panel recommends that ABO D CcEe K-matched RBCs are selected for individuals with SCD and β-thalassemia, even in the absence of alloantibodies, to reduce the risk of alloimmunization. In patients with SCD and β-thalassemia who have developed clinically significant alloantibodies, selection of RBCs antigen negative to the alloantibody is recommended, if feasible. In these patients, selection of more extended phenotype-matched RBCs will likely reduce the risk of further alloimmunization. However, given the limited availability of extended phenotype-matched units, attention should be given to ensure that a delay in transfusion does not adversely affect patient care.
Authors: Sarah J Waldis; Stacey Uter; Donna Kavitsky; Cynthia Flickinger; Sunitha Vege; David F Friedman; Connie M Westhoff; Stella T Chou Journal: Blood Adv Date: 2021-02-09
Authors: Stella T Chou; Mouaz Alsawas; Ross M Fasano; Joshua J Field; Jeanne E Hendrickson; Jo Howard; Michelle Kameka; Janet L Kwiatkowski; France Pirenne; Patricia A Shi; Sean R Stowell; Swee Lay Thein; Connie M Westhoff; Trisha E Wong; Elie A Akl Journal: Blood Adv Date: 2020-01-28