Sickle Cell Disease: Transfusion Support
Publication Date: January 27, 2020
Last Updated: March 14, 2022
Recommendations
Red cell antigen profiling
The ASH guideline panel suggests an extended red cell antigen profile by genotype or serology over only ABO/RhD typing for all patients with SCD (all genotypes) at the earliest opportunity (optimally before the first transfusion). (2⊕ooo)
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Prophylactic red cell antigen matching
The ASH guideline panel recommends prophylactic red cell antigen matching for Rh (C, E or C/c, E/e) and K antigens over only ABO/RhD matching for patients with SCD (all genotypes) receiving transfusions. (1⊕⊕⊕o)
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Prevention of hemolytic transfusion reactions in high-risk patients
The ASH guideline panel suggests immunosuppressive therapy (intravenous immunoglobulin [IVIg], steroids, and/or rituximab) over no immunosuppressive therapy in patients with SCD (all genotypes) with an acute need for transfusion and at high risk for acute hemolytic transfusion reaction or with a history of multiple or life-threatening delayed hemolytic transfusion reactions. (2⊕ooo)
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Management of severe hemolytic transfusion reactions with hyperhemolysis
The ASH guideline panel suggests immunosuppressive therapy (IVIg, steroids, rituximab, and/or eculizumab) over no immunosuppressive therapy in patients with SCD (all genotypes) with a delayed hemolytic transfusion reaction and ongoing hyperhemolysis. (2⊕ooo)
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Transfusion modality in patients with SCD requiring chronic therapy
The ASH guideline panel suggests using automated RCE over simple transfusion or manual RCE in patients with SCD (all genotypes) receiving chronic transfusions. (2⊕ooo)
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Transfusion for patients with SCD and acute chest syndrome
The ASH guideline panel suggests automated RCE or manual RCE over simple transfusions in patients with SCD and severe acute chest syndrome. (2⊕ooo)
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The ASH guideline panel suggests automated RCE, manual RCE, or simple transfusions in patients with SCD and moderate acute chest syndrome. (2⊕ooo)
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Red cell exchange with or without isovolemic hemodilution for chronically transfused patients with SCD
The ASH guideline panel suggests either red cell exchange with isovolemic hemodilution (IHD-RCE) or conventional RCE in patients with SCD (all genotypes) receiving chronic transfusions. (2⊕ooo)
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Transfusion management during pregnancy
The ASH guideline panel suggests either prophylactic transfusion at regular intervals or standard care (transfusion when clinically indicated for a complication or hemoglobin lower than baseline) for pregnant patients with SCD (all genotypes). (2⊕ooo)
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Preoperative transfusion for patients with SCD
The ASH guideline panel suggests preoperative transfusion over no preoperative transfusion in patients with SCD undergoing surgeries requiring general anesthesia and lasting more than 1 hour. (2⊕ooo)
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Screening for transfusional iron overload
The ASH guideline panel suggests iron overload screening by magnetic resonance imaging (MRI; R2, T2*, or R2*) for liver iron content every 1 to 2 years compared with serial monitoring of ferritin levels alone in patients with SCD (all genotypes) receiving chronic transfusion therapy. (2⊕ooo)
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The ASH guideline panel suggests against adding routine iron overload screening by T2* MRI for cardiac iron content compared with serial monitoring of ferritin levels alone in patients with SCD (all genotypes) receiving chronic transfusion therapy. (2⊕ooo)
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Title
Sickle Cell Disease: Transfusion Support
Authoring Organization
American Society of Hematology
Publication Month/Year
January 27, 2020
Last Updated Month/Year
July 5, 2023
External Publication Status
Published
Country of Publication
US
Document Objectives
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.
Target Patient Population
Adults and children with sickle cell disease
Inclusion Criteria
Female, Male, Adolescent, Adult, Child
Health Care Settings
Hospital, Outpatient
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Prevention, Management
Diseases/Conditions (MeSH)
D000755 - Anemia, Sickle Cell, D001803 - Blood Transfusion
Keywords
sickle cell disease, transfusion
Source Citation
Chou ST, Alsawas M, Fasano RM, Field JJ, Hendrickson JE, Howard J, Kameka M, Kwiatkowski JL, Pirenne F, Shi PA, Stowell SR, Thein SL, Westhoff CM, Wong TE, Akl EA. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020 Jan 28;4(2):327-355. doi: 10.1182/bloodadvances.2019001143. PMID: 31985807; PMCID: PMC6988392.