What are the ASH guidelines on transfusion support for patients with sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
  • Print
Answer

ASH 2020 guidelines for transfusion support in patients with SCD recommend 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. [111] The guidelines also include the following suggestions (ie, conditional recommendations based on very low certainty in the evidence):

  • Obtaining an extended red cell antigen profile by genotype (preferred) or serology in all patients with SCD (all genotypes) at the earliest opportunity (optimally before first transfusion). An extended red cell antigen profile includes C/c, E/e, K, Jk a/Jk b, Fy a/Fy b, M/N, S/s at a minimum.
  • In patients with SCD (all genotypes) who are having a delayed hemolytic transfusion reaction and ongoing hyperhemolysis, provide immunosuppressive therapy (IVIg, steroids, rituximab, and/or eculizumab).
  • Automated red cell exchange (RCE) is preferred over simple transfusion or manual RCE in patients with SCD (all genotypes) receiving chronic transfusions.
  • Automated RCE or manual RCE is preferred over simple transfusions in patients with SCD and severe acute chest syndrome.
  • Automated RCE, manual RCE, or simple transfusions may be used in patients with SCD and moderate acute chest syndrome.
  • Use either red cell exchange with isovolemic hemodilution (IHD-RCE) or conventional RCE in patients with SCD (all genotypes) receiving chronic transfusions. (In IHD-RCE, which is available on some automated apheresis devices, the patient undergoes a red cell depletion with concurrent volume replacement before the RCE, with the intent of decreasing the number of red cell units needed for the RCE.) IHD-RCE is not advised for acute indications for RCE or when induction of further anemia during the IHD phase may be generally detrimental (eg, recent history of stroke or transient ischemic attack, severe vasculopathy, or severe cardiopulmonary disease).
  • In pregnant patients with SCD (all genotypes) who have a history of severe SCD-related complications or other high-risk features, prophylactic transfusion at regular intervals may be considered; in other cases, standard care (transfusion when clinically indicated for a complication or hemoglobin lower than baseline) may be provided.
  • Provide preoperative transfusion for patients with SCD undergoing surgeries that require general anesthesia and last longer than 1 hour. The goal for preoperative total hemoglobin levels is > 9 g/dL; RCE transfusion should be used for patients who require preoperative transfusion but have a high hemoglobin level (> 9-10 g/dL) that precludes administration of simple transfusion.
  • To assess liver iron content, iron overload screening by MRI (R2, T2*, or R2*) every 1 to 2 years is preferred over serial monitoring of ferritin levels alone in patients with SCD (all genotypes) receiving chronic transfusion therapy.
  • To assess for cardiac iron content, serial monitoring of ferritin levels alone is preferred over routine iron overload screening by T2* MRI in patients with SCD (all genotypes) receiving chronic transfusion therapy. However, cardiac T2*MRI screening may be considered for the subgroup of patients with SCD with a high iron burden (liver iron content > 15 mg/g [dw]) for 2 years or more, evidence of end organ damage resulting from transfusional iron overload, or evidence of cardiac dysfunction.

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!