Splenic Sequestration and Sickle Cell Disease

Splenic sequestration is a complication of sickle cell disease (SCD). It can lead to severe anemia and is a life-threatening condition.

It is most common among young children with the disease. If your child shows an enlarged spleen or any symptoms of anemia, call your doctor. Early treatment with a blood transfusion can reduce the risk of severe complications. In some cases, surgical removal of the spleen may be necessary.

What is splenic sequestration?

Splenic sequestration is a condition that happens when red blood cells get trapped in the spleen. The spleen is an organ that filters blood and gets rid of old blood cells. Red blood cells getting stuck causes the spleen to enlarge.¹

This also reduces the number of red blood cells circulating in the blood, causing severe anemia. It is life-threatening and should be treated in a hospital.^1,2

An acute episode is often called a splenic sequestration crisis. This is more serious than chronic splenic sequestration. It is most common among young children and is a leading cause of infant mortality. The average age at the first episode is about 1.5 years old. About 30 percent of children with SCD experience acute splenic sequestration events.^3-5

Why does sickle cell disease cause this complication?

Sickle-shaped red blood cells tend to stick to each other and to blood vessel walls. This can happen anywhere in the body and block blood flow to any organ. Blocked blood flow reduces oxygen delivery and leads to acute pain crises for many people with SCD.⁴

Blood flow is especially slow through some parts of the spleen. This makes the spleen a dangerous place for sickle cells to get stuck. When sickle cells get stuck in the spleen, fewer red blood cells are available to circulate in the blood. This can cause severe anemia because the rest of the body cannot get enough oxygen.³

What are symptoms of it?

During a splenic sequestration crisis, the spleen will become hard and enlarged. Your doctor can show you and any caregivers how to feel for the spleen. An enlarged spleen may also cause pain in the left side of the abdomen.⁴

Splenic sequestration can cause severe anemia. If you experience an enlarged spleen or any signs of severe anemia, call your doctor immediately. Symptoms of severe anemia include:^2,4

• Pale skin and lips
• Weakness
• Trouble breathing
• Extreme thirst
• Fast heartbeat
• Poor appetite
• Swelling and pain in the stomach

Splenic sequestration can sometimes happen along with an infection. This is because spleen damage prevents the body from getting rid of certain types of pathogens (organisms that can cause disease and infection). If you experience any symptoms of an infection, such as a fever or chills, call your doctor immediately.³

How is it prevented and treated?

The chance of having a splenic sequestration episode increases after the first episode, especially if it occurs before 1 years old. For people who have had severe episodes or have had many episodes, your doctor may recommend preventive measures, such as:^2,3

• Regular blood transfusions
• Surgical removal of the spleen (splenectomy)

Some reports show that hydroxyurea increases the risk of splenic sequestration crises. Parents of young children treated with hydroxyurea should be extra cautious about splenic sequestration.³

Early and aggressive treatment is necessary, especially for younger children and infants. The first treatment for a splenic sequestration episode is a blood transfusion. This helps the body get oxygen and releases sickle cells trapped in the spleen. Usually, the spleen will return to its normal size and anemia will go away.^3,4

If necessary, your doctor may recommend that you have your spleen surgically removed. You can have a healthy life without a spleen because other organs do many of the same functions. You may take regular antibiotics afterward to reduce the risk of infection.^3,4