American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support

Abstract

Background: Red cell transfusions remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. Guidance for specific indications and administration of transfusion, as well as screening, prevention, and management of alloimmunization, delayed hemolytic transfusion reactions (DHTRs), and iron overload may improve outcomes. Objective: 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. Methods: The American Society of Hematology formed a multidisciplinary panel that was balanced to minimize bias from conflicts of interest and that included a patient representative. The panel prioritized clinical questions and outcomes. The Mayo Clinic Evidence-Based Practice Research Program supported the guideline development process. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to form recommendations, which were subject to public comment. Results: The panel developed 10 recommendations focused on red cell antigen typing and matching, indications, and mode of administration (simple vs red cell exchange), as well as screening, prevention, and management of alloimmunization, DHTRs, and iron overload. Conclusions: The majority of panel recommendations were conditional due to the paucity of direct, high-certainty evidence for outcomes of interest. Research priorities were identified, including prospective studies to understand the role of serologic vs genotypic red cell matching, the mechanism of HTRs resulting from specific alloantigens to inform therapy, the role and timing of regular transfusions during pregnancy for women, and the optimal treatment of transfusional iron overload in SCD.

Anti-C5 antibody treatment for delayed hemolytic transfusion reactions in sickle cell disease

“…[8][9][10][11] The American Society of Hematology (ASH) guidelines include a conditional recommendation for the use of anti-C5 antibodies in patients with SCD presenting DHTR and ongoing hyperhemolysis, based on currently very low levels of certainty. 12 This retrospective study focuses on the biological and clinical findings and the effects of anti-C5 therapy on DHTR, for patients treated between 2013 and 2019 who experienced particularly severe DHTR.…”

“…These findings consolidate the recommendation in the ASH guidelines to use anti-C5 antibody in patients with SCD and ongoing hyperhemolysis. 12 Other anti-complement drugs may also be useful for treatment in this context. A prospective clinical trial would be required to determine whether all DHTR patients would benefit from anti-C5 therapy or whether such treatment is beneficial only for the most severe clinical presentations.…”

Peripartum hyperhemolysis prophylaxis and management in sickle cell disease: A case report and narrative review

“…According to guidelines, RBC units were matched to clinically significant antigens including Rh, Kell, Duffy, Kidd, and MNS. 5,6 Her first exchange transfusion involved 5 units with a pretransfusion Hb level of 9.1 g/dL. After transfusion, there was symptomatic relief and Hb level was 10 g/dL with electrophoresis showing a HbA fraction of 67% and a HbS fraction of 29% (from 88%).…”

“…21 Guidelines from the American Society of Hematology suggest that a chronic transfusion protocol can be considered in pregnant SCD patients with a history of SCD-related complications, additional risk factors contributing to a high-risk pregnancy and those with SCD complications occurring in the pregnancy. 5 Transfusion programs should target a pretransfusion Hb greater than 7.0 g/dL and sickle Hb less than 50%. 5 Transfusion is considered in pregnant SCD patients as concerns have been raised that exposure to HU may result in birth defects or abnormalities of fetal and postnatal growth.…”

“…5 Transfusion programs should target a pretransfusion Hb greater than 7.0 g/dL and sickle Hb less than 50%. 5 Transfusion is considered in pregnant SCD patients as concerns have been raised that exposure to HU may result in birth defects or abnormalities of fetal and postnatal growth. 22 HU is generally not recommended in pregnancy due to its mutagenic, teratogenic, and carcinogenic effects seen in animal studies.…”