Stella T Chou1, Mouaz Alsawas2, Ross M Fasano3, Joshua J Field4, Jeanne E Hendrickson5,6, Jo Howard7,8, Michelle Kameka9, Janet L Kwiatkowski1, France Pirenne10, Patricia A Shi11, Sean R Stowell3, Swee Lay Thein12, Connie M Westhoff13, Trisha E Wong14, Elie A Akl15. 1. Division of Hematology, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA. 2. Mayo Clinic Evidence-Based Practice Research Program, Mayo Clinic, Rochester, MN. 3. Center for Transfusion and Cellular Therapy, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA. 4. Department of Medicine, Medical College of Wisconsin, Milwaukee, WI. 5. Department of Laboratory Medicine and. 6. Department of Pediatrics, Yale University School of Medicine, New Haven, CT. 7. Department of Haematological Medicine, King's College London, London, United Kingdom. 8. Department of Haematology, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom. 9. Nicole Wertheim College of Nursing and Health Sciences, Florida International University, Miami, FL. 10. INSERM-U955, Laboratory of Excellence, French Blood Establishment, Créteil, France. 11. New York Blood Center, New York, NY. 12. Sickle Cell Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD. 13. Laboratory of Immunohematology and Genomics, New York Blood Center, New York, NY. 14. Division of Hematology/Oncology, Department of Pediatrics, Oregon Health and Science University, Portland, OR; and. 15. Department of Internal Medicine, American University of Beirut, Beirut, Lebanon.
Abstract
BACKGROUND: Red cell transfusions remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. Guidance for specific indications and administration of transfusion, as well as screening, prevention, and management of alloimmunization, delayed hemolytic transfusion reactions (DHTRs), and iron overload may improve outcomes. OBJECTIVE: Our objective was to develop evidence-based guidelines to support patients, clinicians, and other healthcare professionals in their decisions about transfusion support for SCD and the management of transfusion-related complications. METHODS: The American Society of Hematology formed a multidisciplinary panel that was balanced to minimize bias from conflicts of interest and that included a patient representative. The panel prioritized clinical questions and outcomes. The Mayo Clinic Evidence-Based Practice Research Program supported the guideline development process. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to form recommendations, which were subject to public comment. RESULTS: The panel developed 10 recommendations focused on red cell antigen typing and matching, indications, and mode of administration (simple vs red cell exchange), as well as screening, prevention, and management of alloimmunization, DHTRs, and iron overload. CONCLUSIONS: The majority of panel recommendations were conditional due to the paucity of direct, high-certainty evidence for outcomes of interest. Research priorities were identified, including prospective studies to understand the role of serologic vs genotypic red cell matching, the mechanism of HTRs resulting from specific alloantigens to inform therapy, the role and timing of regular transfusions during pregnancy for women, and the optimal treatment of transfusional iron overload in SCD.
BACKGROUND: Red cell transfusions remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. Guidance for specific indications and administration of transfusion, as well as screening, prevention, and management of alloimmunization, delayed hemolytic transfusion reactions (DHTRs), and iron overload may improve outcomes. OBJECTIVE: Our objective was to develop evidence-based guidelines to support patients, clinicians, and other healthcare professionals in their decisions about transfusion support for SCD and the management of transfusion-related complications. METHODS: The American Society of Hematology formed a multidisciplinary panel that was balanced to minimize bias from conflicts of interest and that included a patient representative. The panel prioritized clinical questions and outcomes. The Mayo Clinic Evidence-Based Practice Research Program supported the guideline development process. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to form recommendations, which were subject to public comment. RESULTS: The panel developed 10 recommendations focused on red cell antigen typing and matching, indications, and mode of administration (simple vs red cell exchange), as well as screening, prevention, and management of alloimmunization, DHTRs, and iron overload. CONCLUSIONS: The majority of panel recommendations were conditional due to the paucity of direct, high-certainty evidence for outcomes of interest. Research priorities were identified, including prospective studies to understand the role of serologic vs genotypic red cell matching, the mechanism of HTRs resulting from specific alloantigens to inform therapy, the role and timing of regular transfusions during pregnancy for women, and the optimal treatment of transfusional iron overload in SCD.
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