From the Guidelines
Red blood cell exchanges for patients with sickle cell disease should be performed every 3-6 weeks in chronic transfusion programs, with the frequency adjusted based on pre-procedure hemoglobin S (HbS) levels, as recommended by the American Society of Hematology 2020 guidelines 1. The goal of these exchanges is to maintain HbS levels below 30% to prevent sickle cell-related complications, while minimizing iron overload and alloimmunization risks.
- The procedure involves removing the patient's sickle cells and replacing them with normal donor red blood cells, which is more effective than simple transfusions for rapidly reducing HbS concentration while avoiding volume overload and excessive iron accumulation 1.
- Automated red blood cell exchange (RCE) is preferred over manual RCE, as it can more rapidly reduce HbS levels, and special equipment and trained staff are needed for automated RCE 1.
- Patients should be monitored for transfusion reactions, iron levels, and development of alloantibodies throughout their chronic exchange program.
- A pre- and postprocedure complete blood count and hemoglobin fractionation should be obtained to maximize procedure safety and efficacy 1. The American Society of Hematology 2020 guidelines suggest that automated RCE likely reduces the risk for iron overload and increases the probability of achieving the desired preprocedure HbS suppression without increasing the risk of other adverse events 1.
From the Research
Red Blood Cell Exchanges in Chronic Programs
- Red blood cell exchanges are typically performed every 3-6 weeks in chronic programs, with the frequency adjusted based on pre-procedure HbS levels 2, 3.
- The goal of these exchanges is to reduce HbS levels and prevent complications such as acute chest syndrome and stroke 2, 4.
- The management of sickle cell disease varies greatly among patients, and some may benefit from minimal therapy and infrequent transfusions, while others are transfusion-dependent 2.
Indications for Red Blood Cell Exchange
- Red blood cell exchange is indicated to prevent or treat complications arising from the presence of HbS, such as acute chest syndrome and stroke 3, 4.
- The procedure can help reduce HbS levels rapidly, which may be required in certain critical conditions 2, 3.
- The target HbS level is typically not more than 30%, and the target hematocrit level is less than 30% 3.
Frequency of Red Blood Cell Exchanges
- The frequency of red blood cell exchanges can vary depending on the individual patient's needs and response to treatment 2, 3.
- Regular long-term red blood cell transfusions may be necessary for managing chronic chest complications in sickle cell disease 5, 6.
- However, there is a need for randomized controlled trials to determine the optimal frequency and duration of red blood cell exchanges in chronic programs 5, 6.