Blood transfusion for sickle cell disease

Last updated Aug. 16, 2024, by Marisa Wexler, MS
Fact-checked by Joana Carvalho, PhD

What is a blood transfusion?

A blood transfusion is a medical procedure through which healthy blood cells or blood components are transferred from a healthy donor to someone in need. It may be used to help treat, mitigate, or prevent the symptoms and chronic complications of sickle cell disease (SCD) in both children and adults.

SCD is a disorder that impacts red blood cells, which are primarily responsible for carrying oxygen throughout the body. SCD is caused by genetic mutations that lead to the production of an abnormal form of hemoglobin — the protein that red blood cells use to carry oxygen — with red blood cells taking on the sickle-like shape that characterizes the disease.

Sickled red blood cells are more likely to die prematurely and to get trapped inside blood vessels, blocking blood flow. As a result, the misshapen red blood cells in people with SCD can’t carry and deliver oxygen to tissues as efficiently as those of healthy people.

By delivering healthy red blood cells to SCD patients, blood transfusions can help improve blood flow and oxygen delivery to tissues and organs throughout the body. This can ease SCD symptoms, prevent complications, and improve patients’ quality of life.

When are blood transfusions used in SCD?

According to blood transfusion guidelines in sickle cell disease, there are a few instances where a red blood cell transfusion may be indicated. Transfusion is one of the options for treatment for SCD, and it may be used on its own or in combination with other therapies, depending on each individual’s specific needs.

In some cases, an acute transfusion is indicated, meaning one or several infusions are given to address a serious and ongoing medical concern. Acute blood transfusions may be used in SCD:

as part of treatment for anemia (low red blood cell count).
to help prevent and/or manage stroke, particularly in children and adolescents, as well as related disorders characterized by blood flow impairment in the brain.
to help treat acute chest syndrome, a complication of SCD caused by blood vessel blockage in the lungs that can be life-threatening.
as part of treatment for acute hepatic and splenic sequestration, which occur when red blood cells get trapped inside the liver and spleen, causing both organs to enlarge.
as part of management for some forms of organ damage or multiorgan failure.
before surgery to reduce the risk of SCD-related complications during operations lasting more than one hour and requiring general anesthesia.
during pregnancy to reduce or manage SCD-related complications in women with a history of severe SCD complications or with additional high-risk pregnancy features.

In other cases, people with SCD may be given chronic transfusions, where infusions are given routinely over the course of months or years. Indications for chronic blood transfusion in sickle cell disease may include:

reducing the risk of stroke in adults who have previously experienced a stroke or who have risk factors for stroke.
lowering the risk of vaso-occlusive crises (VOCs), painful episodes caused by clumped sickle cells restricting blood flow, in patients with recurrent VOCs.

How does the procedure work?

Two main types of blood transfusions are typically used in SCD:

Simple transfusion, which involves the administration of new healthy red blood cells into the patient’s bloodstream.
Exchange transfusion, where the patient’s own red blood cells are removed during or shortly before new healthy red blood cells are transferred.

Generally, simple transfusions are preferred when patients have substantial anemia, whereas exchange transfusions are recommended for patients with minimal anemia, with the goal of maintaining a roughly normal count of healthy red blood cells in the body.

Exchange transfusion also is generally preferred for the prevention and management of stroke and other blood flow problems, where it is beneficial to reduce the number of red blood cells carrying mutated hemoglobin.

Blood transfusions are given via infusions into the bloodstream. This is usually done through a needle inserted into a vein in the arm, though other setups are possible. For example, patients receiving blood transfusions regularly may be equipped with a port for easier access.

In an exchange transfusion, which requires specialized equipment and staff to perform and is typically more costly, the patient’s blood may be removed by the same machine doing the transfusion at the same time and in an automated manner. Alternatively, it can be done through a manual process, in which the exchange is conducted in cycles.

Throughout the transfusion, patients will routinely have their pulse and blood pressure monitored. A typical transfusion lasts from one to three hours, though times can vary depending on the specific situation.

Because blood transfusion in sickle cell disease  aims to increase the number of red blood cells carrying healthy hemoglobin, autologous transfusion — a procedure in which blood is collected from a patient and stored to be transfused back to the same individual at a later date — is not typically used in SCD. Instead, people with SCD rely on donated blood products stored via blood banking.

Benefits of blood transfusions

In the short term, blood transfusions allow more oxygen to be delivered to tissues, help prevent blood flow blockage, and reduce the body’s need to make new blood cells. Increased oxygen delivery also can reduce the risk of serious organ damage.

Transfusions can help to alleviate sickle cell symptoms like fatigue and pain, which are caused mainly by poor oxygen delivery to body tissues. Long-term, chronic transfusions can help lower the risk of serious complications, such as stroke and acute chest syndrome, in children and adults with SCD.

Risks and considerations

Several considerations should be taken into account both before and after a blood transfusion.

Pre-transfusion considerations, including blood type matching, are key to reduce the chances of acute complications related to the procedure. Patients should also be closely monitored after receiving a blood transfusion to ensure that, if a problematic reaction occurs, it can be dealt with immediately.

Transfusion reactions

A person’s blood type refers to certain molecular markers that are present on their blood cells. The immune system is wired to attack anything that it fails to recognize as being a normal part of the body. Therefore, if a patient receives a transfusion with blood cells bearing a marker that isn’t on their own cells, their immune system may attack the transfused cells.

Such transfusion reactions can cause patients to experience symptoms including rash, itching, chills, fever, pain, and, in more severe cases, shortness of breath. While possible, it is extremely rare that transfusion reactions are fatal. Indeed, the odds of dying from a transfusion reaction, which are posed to be at approximately 1 in 2 million, are lower than the odds of being struck by lightning (less than 1 in 1 million).

When a life-threatening reaction occurs or is suspected, the transfusion should be stopped immediately and appropriate supportive treatment given. Patients who have experienced these types of reactions may also be treated with immune-suppressing medications to reduce the risk of similar reactions in future blood transfusions, or they may need to find alternative forms of treatment.

As transfusions are typically an important part of long-term management for people with SCD, the American Society of Hematology recommends that everyone with SCD undergo detailed blood typing as early as possible, ideally before a first transfusion is needed, to ensure they can have access to appropriate blood products when necessary.

Iron overload

Iron overload is also among the list of potential complications of blood transfusion in sickle cell disease.

Because blood cells contain a lot of iron, blood transfusions can cause iron levels in the body to rise and become excessively high. This is known as iron overload, and it can potentially lead to major organ damage. The condition is generally prevented or managed with iron chelators — medications that are specifically designed to bind to and neutralize iron, helping to eliminate it from the body.

Iron overload is more likely to occur in patients receiving simple transfusions than in those receiving exchange transfusions.

Transmission of infections

In the past, there was a notable risk of blood-transmitted viral infections, such as hepatitis or HIV, in people receiving blood transfusions.

With modern screening techniques, however, the risk of contracting infections from blood products is extremely low. Estimates indicate that the odds of contracting such viral infections nowadays are less than 1 in 1 million.

Recovery and long-term care

After a blood transfusion, patients generally are advised to rest for a day or two as part of post-transfusion care. They usually can go home as soon as the transfusion is finished, unless additional treatment or monitoring is necessary.

During a blood transfusion, and in the days and weeks after the procedure, patients should immediately contact their healthcare providers if they begin experiencing any of the following symptoms, which may be signs of transfusion reactions:

bleeding, pain, or new bruising at the infusion site
fever, chills, or feeling cold and clammy
urine that’s unusually dark or reddish
an abnormally fast heartbeat
chest pain, wheezing, or difficulty breathing
severe back pain
itching, rash, or hives
nausea or vomiting
headache or dizziness
jaundice (yellowing of the skin and eyes).

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