A new review discussing the risk factors, mitigation strategies, and management recommendations for patients with sickle cell disease (SCD) has been published in Transfusion and Apheresis Science.

The authors of the review are Margo R. Rollins, MD, from Children’s Healthcare of Atlanta and Emory University School of Medicine, Department of Pediatrics, Aflac Cancer and Blood Disorders Center in Atlanta, Georgia, and Stella T. Chou, MD, from the Children’s Hospital of Philadelphia in Pennsylvania.

Drs. Rollins and Chou concluded that “cautious and thoughtful treatment can ameliorate or prevent recurring events or long-term consequences of red cell transfusion exposure.”

The highlights from the review are as follows:

  • alloimmunization and iron overload are risk factors for red blood cell transfusion in SCD
  • prophylactic Rh and K matching of red blood cells are recommended
  • RH variation among African patients and donors contributes to Rh alloimmunization
  • immunosuppression can be tailored for severe hemolytic transfusion reactions.

These findings are important because patients with SCD often need blood transfusions, which put them at risk of transfusion-related adverse events including alloimmunization, hemolytic transfusion reactions, hyperviscosity, and transfusion-associated iron overload.

Read more about the treatment of SCD 

SCD is a rare inherited blood disorder characterized by the sickling and early death of red blood cells due to alterations in the structure of hemoglobin. This results in the inefficient transportation of oxygen to the tissues and the obstruction of small blood vessels, leading to a condition known as vaso-occlusive crisis. 

The vast majority of patients with SCD need at least 1 blood transfusion in their life. A blood transfusion can improve blood flow, increase the oxygen-carrying capacity of the blood, and prevent complications such as severe anemia and acute organ damage. However, transfusion therapy is associated with several risks, including infections, alloimmunization, and hemosiderosis, and must be performed with caution.

Reference

Rollins MR, Chou ST. Adverse events of red blood cell transfusions in patients with sickle cell disease. Transfus Apher Sci. Published online August 29, 2022. doi:10.1016/j.transci.2022.103557