Sickle Cell Disease: Transfusion Support

Publication Date: January 27, 2020

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)
312101

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)
312101

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)
312101

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)
312101

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)
312101

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)
312101
The ASH guideline panel suggests automated RCE, manual RCE, or simple transfusions in patients with SCD and moderate acute chest syndrome. (2⊕ooo)
312101

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)
312101

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)
312101

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)
312101

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)
312101
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)
312101

Recommendation Grading

Disclaimer

Overview

Title

Sickle Cell Disease: Transfusion Support

Authoring Organization

Publication Month/Year

January 27, 2020

Supplemental Implementation Tools

Document Type

Guideline

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.