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Acute Spleen Enlargement (Splenic Sequestration)

What is spleen enlargement?

Spleen enlargement, also known as splenic sequestration, is swelling or growth of the spleen. It is caused by sickle cell disease.

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

  • Fight infection
  • Create and filter blood cells

There are 2 types: acute and chronic.

  • Chronic enlargement may not cause problems. Your doctor records the size of the spleen at each visit to make sure it is not getting larger. The condition usually occurs in older children and adults with sickle cell disease.
  • Acute enlargement is a sudden increase of spleen size that can be life-threatening. In sickle cell disease, this can happen at any age. But it normally occurs in infants and young children. The condition happens when sickled red blood cells get trapped in the spleen, causing it to grow. You may hear the care team use the term “acute spleen” to describe this condition.

Sickle cell disease affects the red blood cells causing them to sickle or become banana shaped. The main purpose of red blood cells is to deliver oxygen to the body. Sickled red blood cells stick together and slow the flow of oxygen to the tissues.

When sickled red blood cells are trapped in the spleen, the rest of the body does not get enough oxygen. If not treated, an acute spleen can cause the body to go into shock. It is an emergency and may be life-threatening.

Symptoms of acute spleen

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 pain: The spleen becomes hard and enlarged and extends below the ribcage. This may cause pain. Your doctor can show you how to feel the spleen.
  • Lack of energy: Patients may lack energy to play. They appear tired and are hard to wake up.

Treatment for acute spleen

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

The risk for another episode of acute spleen is high. The doctor may recommend removal of the spleen if the first case was severe or if a second case occurs.

Living without a spleen

Other organs in the body perform the same function as the spleen. A person can have a healthy life without a spleen. The doctor may prescribe daily penicillin for life to help decrease the risk of infection.

Key Points

  • Splenic sequestration is enlargement of the spleen. There are 2 types common to sickle cell disease: acute and chronic.
  • Acute spleen is life-threatening. Seek medical help if your child shows symptoms of anemia, spleen hardness or enlargement, or severe fatigue.
  • The risk is high for a repeat case. The doctor may recommend removing the spleen.

Reviewed: September 2022