What is the role of blood transfusion in Sickle Cell Disease (SCD)?

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Last updated: March 6, 2025 • View editorial policy

From the Guidelines

Blood transfusions are a crucial treatment for Sickle Cell Disease (SCD), particularly for preventing recurrent ischemic stroke, and should be administered to reduce hemoglobin S to <30% of total hemoglobin in patients with prior ischemic stroke or TIA. The primary goal of blood transfusion in SCD is to decrease the concentration of hemoglobin S, thereby reducing the risk of sickling episodes and associated complications, such as stroke. According to the 2021 guideline for the prevention of stroke in patients with stroke and transient ischemic attack 1, chronic blood transfusion is recommended for patients with SCD and prior ischemic stroke or TIA to prevent recurrent ischemic stroke. Key considerations in transfusion therapy include:

  • The standard transfusion dose is typically 10-15 mL/kg of packed red blood cells, although this may vary based on the patient's condition and hemoglobin levels.
  • Extended phenotype matching is essential to reduce the risk of alloimmunization.
  • Potential complications of chronic transfusion include iron overload, which requires chelation therapy with medications like deferasirox or deferiprone when patients have received multiple transfusions. The American Society of Hematology 2020 guidelines for SCD also suggest automated red cell exchange (RCE) over manual RCE or simple transfusion for patients requiring chronic transfusions, due to reduced iron overload and improved HbS suppression 2. However, it is crucial to weigh the benefits of transfusion against potential risks, such as hyperhemolysis, and to carefully manage these complications when they occur 3. Overall, blood transfusions play a vital role in the management of SCD, particularly in preventing stroke and other complications, and should be used in accordance with established guidelines and patient-specific considerations.

From the Research

Role of Blood Transfusion in Sickle Cell Disease (SCD)

  • Blood transfusion has been a beneficial therapy for SCD since the 1950s, with advances reducing side effects and increasing indications for transfusion 4
  • The primary goal of transfusion is to increase blood oxygen carrying capacity and decrease the relative amount of hemoglobin S (HbS) to hemoglobin A (HbA) to mitigate vaso-occlusion in small blood vessels 5

Indications for Transfusion

  • Transfusion is used to prevent stroke, treat acute chest syndrome, and manage perioperative complications in SCD patients 4, 6, 7
  • It is also used to optimize pregnancy outcomes and reduce the risk of neurological disease 7
  • Transfusion may be utilized in efforts to intervene in the evolution of potentially life-threatening complications of SCD, such as acute stroke and severe acute anemia 5

Methods of Transfusion

  • Simple transfusion, partial manual RBC exchange, and automated RBC exchange by apheresis are used to achieve transfusion goals 5
  • The choice of transfusion method depends on the individual patient's needs and the specific clinical scenario 8

Complications and Considerations

  • Transfusion is associated with numerous complications, including iron overload, alloimmunization, and transfusion reactions 4, 6
  • Blood bank considerations, such as adequate RBC antigen matching, are important to mitigate the risk of RBC alloimmunization 5
  • Individualized treatment plans and continuous evaluation of clinical indications are essential components of patient care 5, 7

References

Research

Current issues in blood transfusion for sickle cell disease.

Current opinion in pediatrics, 2009

Research

Transfusion support for patients with sickle cell disease.

Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis, 2022

Research

Blood transfusion for preventing stroke in people with sickle cell disease.

The Cochrane database of systematic reviews, 2002

Research

Indications for transfusion in the management of sickle cell disease.

Hematology. American Society of Hematology. Education Program, 2021

Research

Sickle cell disease: when and how to transfuse.

Hematology. American Society of Hematology. Education Program, 2016

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.