Transfusion support in sickle cell disease: ASH 2020 guidelines

USA: The American Society of Hematology (ASH) has released its guideline on transfusion support for management of patients with sickle cell disease (SCD). 

Transfusion support remains a key intervention in the management of patients with sickle cell disease (SCD). Red cell transfusions are used in the acute and chronic management of many complications related to SCD, but are not without adverse effects, including alloimmunization and iron overload. Specific indications, mode of red cell administration and transfusion-related complications continue to pose significant challenges for patients and providers, and are the focus of these guidelines. The American Society of Hematology (ASH) guideline panel addressed specific questions related to the following areas: extent of red cell antigen typing and matching, transfusion indications and mode of administration (simple vs red cell exchange [RCE] transfusion), prevention and management of alloimmunization and delayed hemolytic transfusion reactions (DHTRs), and screening for iron overload.

These guidelines are based on updated and original systematic reviews of evidence conducted by the Mayo Clinic Evidence-Based Practice Research Program. The panel followed best practice for guideline development recommended by the Institute of Medicine and the Guidelines International Network.1-4 The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach5-11 to assess the certainty of the evidence and formulate recommendations.

Following are the major recommendations: 

• 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)
• 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
• 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
• 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
• The ASH guideline panel suggests using automated RCE over simple transfusion or manual RCE in patients with SCD (all genotypes) receiving chronic transfusions
• The ASH guideline panel suggests automated RCE or manual RCE over simple transfusions in patients with SCD and severe acute chest syndrome
• The ASH guideline panel suggests automated RCE, manual RCE, or simple transfusions in patients with SCD and moderate acute chest syndrome
• 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
• 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)
• 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
• 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
• 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.

For more details click on the link: https://doi.org/10.1182/bloodadvances.2019001143