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Practice Guideline
. 2020 Jan 28;4(2):327-355.
doi: 10.1182/bloodadvances.2019001143.

American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support

Stella T Chou  1 , Mouaz Alsawas  2 , Ross M Fasano  3 , Joshua J Field  4 , Jeanne E Hendrickson  5   6 , Jo Howard  7   8 , Michelle Kameka  9 , Janet L Kwiatkowski  1 , France Pirenne  10 , Patricia A Shi  11 , Sean R Stowell  3 , Swee Lay Thein  12 , Connie M Westhoff  13 , Trisha E Wong  14 , Elie A Akl  15
Affiliations
Practice Guideline

American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support

Stella T Chou et al. Blood Adv. .

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.

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Conflict of interest statement

Conflicts of interest of all participants were managed through disclosure, panel composition, and recusal, according to recommendations of the Institute of Medicine and the Guidelines International Network. Participants disclosed all financial and nonfinancial interests relevant to the guideline topic. ASH staff and the ASH Guideline Oversight Subcommittee reviewed the disclosures and composed the guideline panel to include a diversity of expertise and perspectives and avoid a majority of the panel having the same or similar conflicts. The greatest attention was given to direct financial conflicts with for-profit companies that could be directly affected by the guidelines. A majority of the panel, including the cochairs, had no such conflicts. None of the Mayo-affiliated researchers who contributed to the systematic evidence reviews or who supported the guideline development process had any such conflicts.

References

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