Acute Spleen Enlargement (Splenic Sequestration)

What is acute spleen enlargement?

Acute spleen enlargement is a sudden increase of spleen size that can be life-threatening. It may also be called a splenic sequestration crisis or acute spleen. In sickle cell disease, this can happen at any age. But it normally happens in infants and young children.

The spleen is an organ on the left side of the body under the ribcage. It is about the size of a fist. The spleen works to:

• Fight infection
• Make white blood cells
• Create, store, and filter blood

If not treated, an acute spleen can cause the body to go into shock. It is an emergency. Get medical help right away if your child has symptoms of acute spleen.

Symptoms of acute spleen

Signs and symptoms of acute spleen include:

• Anemia: Signs include pale skin, weakness, trouble breathing, and fast heartbeat. A person with sickle cell disease develops anemia quickly because not enough blood is circulating through the body.
• Hardness or belly pain: The spleen becomes hard and enlarged so that it extends below the ribcage. It may cause pain if you press on the area. Your care team can show you how to feel your child’s spleen to make sure it is not enlarged.
• Lack of energy: Your child may lack energy to play and be cranky. They may seem tired or be hard to wake up.
• Sometimes, fever or infection may occur.

If your child is having trouble breathing, becomes very pale or limp, or is not responding, call 911.

Causes of acute spleen

Acute spleen happens when too many sickled blood cells get trapped in the spleen, causing the spleen to get larger. The blood in the spleen becomes separated (sequestered) from the blood in the body. As a result, the rest of the body does not get enough oxygen.

Some people with sickle cell disease may have chronic spleen enlargement. This may not cause problems. Chronic spleen enlargement usually occurs in older children and adults with sickle cell disease.

Diagnosis of acute spleen

Acute spleen is diagnosed based on your child’s medical history, a physical exam, and lab tests. 

To diagnose acute spleen, your child’s care team may also perform an ultrasound if the spleen cannot easily be felt.

Treatment of acute spleen

Treatments for acute spleen include red blood cell transfusions, IV fluids, and antibiotics.

Red blood cell transfusion: The first treatment for acute spleen is red blood cell transfusion. This gives much-needed oxygen to the cells and releases the sickled red blood cells that are trapped in the spleen. As this happens, the spleen gets smaller, and the anemia goes away.

Surgery to remove the spleen: If your child has had acute spleen, the risk of getting it again is high. Your care team may recommend the spleen be removed (splenectomy) if your child has had severe or repeated occurrences of acute spleen. Your child can have a healthy life without a spleen. But they will be more likely to get infections. Your care team may recommend vaccines and prescribe daily penicillin (an antibiotic) for life to help decrease the risk of infection.

Watch for symptoms of acute spleen and keep all medical appointments. Your care team will monitor your child for acute or chronic spleen enlargement.

Your care team might recommend a medical ID bracelet for your child. They may also advise limiting high-contact sports or physical activity that have a risk of injuring the spleen.

 Questions to ask your care team

• What are the symptoms of acute spleen?
• What are the complications of acute spleen?
• What are the treatment options for acute spleen?
• Is my child at higher risk for acute spleen?
• What emergency warning signs should I watch for?
• How do I feel for my child’s spleen?

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