Blood transfusions for sickle cell disease

Treatment Overview

During a blood transfusion, a person (the recipient) receives healthy blood from another person (the donor). The donated blood is carefully screened for diseases before it is used. Before receiving a blood transfusion, the recipient's blood is analyzed closely (using blood type) to make sure the donor blood is a close match to the recipient's.

Blood is transfused into an arm vein slowly over 1 to 4 hours (except in an emergency when blood is transfused more quickly).

Blood transfusions can:

  • Treat a sudden or short-term condition related to sickle cell disease.
  • Treat severe complications of sickle cell disease.
  • Prevent complications of sickle cell disease.
  • Lower the risk of stroke in infants and children.

Blood transfusions aren't usually used to treat uncomplicated painful events or mild to moderate anemia.

What To Expect After Treatment

Following a blood transfusion, doctors keep a close watch on the person for any negative reactions.

  • A transfusion reaction, resulting from mismatched blood type, may occur immediately or days later (5 to 20 days after transfusion). An acute transfusion reaction ranges from mild (fever, chills, and rash) to severe (shock, severe anemia, painful event, and death).
  • A person receiving repeat blood transfusions may develop antibodies to the donor blood (called alloimmunization). Alloimmunization makes repeated transfusions more difficult. It occurs in about 1 out of 4 people with sickle cell disease who get frequent transfusions.1

Hospital staff will also check for iron buildup in the body (iron overload). This can develop when a person gets many transfusions.

Why It Is Done

A blood transfusion lowers the amount of hemoglobin S red blood cells in the body. When there are fewer sickled hemoglobin S cells in the bloodstream, they are less likely to build up and block blood vessels.

Blood transfusion also increases the number of normal red blood cells in the body, increasing the supply of oxygen to the body.

Anemia

Some sudden complications of sickle cell disease cause the body's red blood cell count to drop to life-threatening levels (severe anemia). When severe, these conditions (including splenic sequestration, acute chest syndrome, and aplastic crisis) can be fatal if not treated with blood transfusions.

Chronic severe anemia from kidney failure may need treatment with periodic blood transfusions.

Surgery

After having general anesthesia and surgery, people with sickle cell disease are at risk for sickling-related problems and acute chest syndrome. Blood transfusions before surgery can prevent or treat these complications.

Red blood cell sickling-related complications (vaso-occlusion)

Blood transfusions can treat acute chest syndrome and leg ulcers.

Frequent blood transfusions may help prevent strokes in children who already had a stroke or are at high risk for a first stroke.2

How Well It Works

Blood transfusion is currently the single most effective and proven treatment for some severe complications of sickle cell disease.3

Blood transfusions reduce the risk of some complications of sickle cell disease and improve symptoms of severe anemia. Using repeat blood transfusions for 3 to 5 years can reduce the number of repeat strokes in children who have had a stroke.

Risks

A person receiving many blood transfusions will gradually collect too much iron in the body (iron overload). Very high levels of iron can lead to hemochromatosis, which can be fatal if untreated.

After 10 to 20 blood transfusions, iron chelation treatment can help rid the body of excess iron.

Donated blood is carefully screened for diseases before it is used. But it's remotely possible that donor blood may be contaminated with an infectious disease (such as HIV or hepatitis).

What To Think About

If you have repeated blood transfusions, you'll have routine testing for iron buildup in your body.

Though blood transfusions are an effective treatment for sickle cell disease complications, they are only used selectively. The risks of hemochromatosis and alloimmunization from repeat transfusions makes this procedure more suitable for severe and high-risk conditions.

Doctors and researchers continue to weigh the benefits against the risks of preventive blood transfusions. Repeat blood transfusion treatment for 3 to 5 years can reduce the number of repeat strokes in children who have sickle cell disease. It helps prevent a second stroke in most children. But some children who get repeat transfusions will still have a second stroke.

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References

Citations

  1. Steinberg MH (2008). Sickle cell disease and associated hemoglobinopathies. In L Goldman, D Ausiello, eds., Cecil Medicine, 23rd ed., pp. 1217–1226. Philadelphia: Saunders Elsevier.
  2. Benz EJ (2008). Disorders of hemoglobin. In AS Fauci et al., eds., Harrison's Principles of Internal Medicine, 17th ed., pp. 635–643. New York: McGraw-Hill Medical.
  3. Wang WC (2004). Sickle cell anemia and other sickling syndromes. In JP Greer et al., eds., Wintrobe's Clinical Hematology, 11th ed., pp. 1264–1311. Philadelphia: Lippincott Williams and Wilkins.

Last Updated: December 9, 2008

Author: Debby Golonka, MPH

Medical Review: Anne C. Poinier, MD - Internal Medicine & Martin Steinberg, MD - Hematology

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