From the Guidelines
Exchange transfusion in sickle cell disease (SCD) is indicated for several acute and chronic complications, including acute stroke or neurological symptoms, acute chest syndrome unresponsive to standard therapy, multi-organ failure, severe acute intrahepatic sequestration, and preparation for major surgery requiring general anesthesia. The primary goal of exchange transfusion is to reduce the percentage of hemoglobin S to below 30% while maintaining a total hemoglobin level of 10-11 g/dL, thereby improving oxygen delivery to tissues, reducing blood viscosity, and preventing further sickling 1.
Indications for Exchange Transfusion
- Acute stroke or neurological symptoms
- Acute chest syndrome unresponsive to standard therapy
- Multi-organ failure
- Severe acute intrahepatic sequestration
- Preparation for major surgery requiring general anesthesia
- Priapism lasting more than 4 hours unresponsive to initial interventions
- Severe acute splenic sequestration
- Recurrent or refractory vaso-occlusive crises despite hydroxyurea therapy
Chronic Management
Exchange transfusion is used for primary and secondary stroke prevention in children with abnormal transcranial Doppler velocities, recurrent acute chest syndrome, and frequent severe painful crises affecting quality of life 1.
Procedure
Exchange transfusion is preferred over simple transfusion for many acute complications because it reduces the risk of hyperviscosity, iron overload, and volume overload while effectively lowering the hemoglobin S percentage 1. Automated RCE can reduce HbS levels more rapidly than manual RCE, and should be considered for patients with rapidly progressive ACS, who do not respond to initial treatment with simple transfusion, or with high pretransfusion hemoglobin levels that preclude simple transfusion 1.
Risks and Complications
Acute and delayed hemolytic transfusion reactions (HTRs) are among the most challenging complications of transfusion support in patients with SCD, particularly as they may have a fatal outcome 1. Recognition of hyperhemolysis is critical, as additional transfusions should be avoided if possible, and treatment with extended matched red cells, IVIg, high-dose steroids, eculizumab, and/or rituximab may be necessary 1.
From the Research
Indications for Exchange Transfusion in Sickle Cell Disease
The following are indications to start exchange transfusion in a sickle cell disease (SCD) patient:
- Acute chest syndrome (ACS) 2, 3, 4, 5
- Stroke 2, 4, 5
- Hepatic/splenic sequestration 6
- Multiorgan dysfunction syndrome (MODS) 6
- Perioperative management of SCD 4, 5
- Prevention of stroke 4, 5
- Treatment of acute chest syndrome 4, 5
- Pulmonary hypertension 5
Exchange Transfusion Techniques
Exchange transfusion can be performed using different techniques, including:
- Red blood cell exchange transfusion (RBCEX) 2, 6
- Therapeutic plasma exchange (TPE) 6
- Manual, double-volume exchange transfusion 3
Goals of Exchange Transfusion
The goals of exchange transfusion in SCD patients include: