The American Society of Hematology (ASH) recently released new clinical guidelines on transfusion support for sickle cell disease (SCD) in a publication in Blood Advances.1

“One therapy that is currently a cornerstone therapy [in SCD] is transfusions. Even though there are many exciting new developments in gene therapy and stem cell transplant, the reality is that many patients with SCD still require transfusions,” said first author of the report and cochair of the ASH guideline panel Stella Chou, MD, of the division of hematology and the apheresis program at the Children’s Hospital of Philadelphia, Pennsylvania.

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The multidisciplinary ASH guideline panel, which included a patient representative, worked with the Mayo Clinic Evidence-Based Practice Research Program to develop evidence-based guidelines to inform patients, clinicians, and other healthcare professionals making decisions regarding transfusion support for SCD and managing transfusion-related complications, including alloimmunization and iron overload. The guidelines were also subject to public comment prior to finalization.

The panel established 10 recommendations based on specific questions (see Table) related to the extent of red cell antigen typing and matching, transfusion indication and mode of administration, prevention and management of alloimmunization and delayed hemolytic transfusion reactions (HTRs), and screening for iron overload.

Table. Prioritized Questions by the ASH Guideline Panel on Transfusion Support in SCD1
1. Should an extended red cell antigen profile be obtained by genotype or serology compared with only ABO/RhD type for patients with SCD?
2. Should prophylactic Rh (C, E, or C/c, E/e)- and K-matched red cells or prophylactic Rh (C, E or C/c, E/e)-matched, K-matched, and extended matched (Jka/Jkb, Fya/Fyb, S/s) red cells, by serologic or genotype-predicted red cell antigen profile, compared with only ABO/RhD-matched red cells be used for patients with SCD receiving transfusions?
3. Should immunosuppressive therapy (IVIG, steroids, and/or rituximab) or no immunosuppressive therapy be used for patients with SCD (all genotypes) with an acute need for transfusion and with a high risk for HTRs?
4. Should immunosuppressive therapy (IVIG, steroids, rituximab, and/or eculizumab) or no immunosuppressive therapy be used for patients with SCD (all genotypes) with ongoing hyperhemolysis (defined as rapid decline of posttransfusion hemoglobin to below the pretransfusion level)?
5. Should automated RCE compared with simple transfusion or manual RCE be used for patients with SCD receiving chronic transfusions?
6. Should automated or manual RCE be used over simple transfusion for patients with SCD and severe acute chest syndrome?
7. Should IHD-RCE or conventional RCE be used for patients with SCD receiving chronic transfusions?
8. Should prophylactic transfusion at regular intervals or standard care (transfusion only when indicated for a complication or exacerbated anemia) be provided to pregnant patients with SCD?
9. Should preoperative transfusion or no preoperative transfusion be used for patients with SCD undergoing surgeries requiring general anesthesia and lasting longer than 1 hour?
10. (a) Should iron overload screening by MRI for liver iron content or serial monitoring of ferritin levels alone be used for patients with SCD receiving chronic transfusion therapy?
(b) Should iron overload screening by MRI for cardiac iron content or serial monitoring of ferritin levels alone be used for patients with SCD receiving chronic transfusion therapy?
Abbreviations: HTS, hemolytic transfusion reactions; IHD-RCE, red cell exchange with isovolemic hemodilution; IVIG, intravenous immunoglobulin; MRI, magnetic resonance imaging; RCE, red cell exchange; SCD, sickle cell disease. 

“Oftentimes, the care that patients with SCD receive from institution to institution might vary somewhat, depending on where they live, and so, part of the goal of the guidelines was to help to make their management more uniform from place to place,” added Dr Chou.

A majority of the recommendations are conditional recommendations due to the paucity of currently available evidence for outcomes of interest.