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Acute splenic sequestration and sickle cell disease

 

What is the spleen?

The spleen is an oval-shaped organ located on the left side of the body. It is protected by the ribcage. The spleen has two functions: it helps the body fight infection, and it helps to create and filter blood cells.

Image of enlarging spleen in a child

Size as the spleen enlarges.

What is splenic sequestration?

Splenic sequestration (see kwes TRAY shun) is enlargement of the spleen. There are two types of splenic sequestration in sickle cell disease— acute and chronic.

Chronic splenic sequestration may not cause problems and the doctor may choose to record the size of the spleen at each visit to make sure it is not getting any larger. Chronic splenic sequestration usually occurs in older children and adults with sickle cell disease.

Acute splenic sequestration is a sudden enlargement of the spleen that can be life-threatening. In sickle cell disease, acute splenic sequestration can happen at any age, but normally it occurs in infants and young children. Acute splenic sequestration happens when sickled red blood cells get trapped in the spleen, causing the spleen to enlarge.

Sickle cell disease affects the red blood cells causing them to sickle or become banana-shaped. The main purpose of red blood cells (RBCs) is to deliver oxygen to the body. Sickled red blood cells stick together and slow the flow of oxygen to the tissues. When sickled RBCs are trapped in the spleen, the rest of

the body does not get enough oxygen. If not treated, acute splenic sequestration can cause the body to go into shock. Acute splenic sequestration is a medical emergency!

What are the symptoms of acute splenic sequestration in a person with sickle cell disease?

A person with sickle cell disease will become anemic quickly because not enough blood is circulating through the body. Signs of severe anemia include pale skin, weakness, trouble breathing, and a fast heartbeat. Also, the spleen will become hard and enlarged and will extend below the ribcage. Your doctor can show you how to feel for the spleen. Splenic sequestration can be very painful. Young infants will not have energy to play and will appear extremely drowsy or lack energy and will be hard to awaken. Acute splenic sequestration is an emergency. Seek medical treatment right away.

What is the treatment for acute splenic sequestration?

The immediate treatment is red blood cell transfusion. This provides the body with much needed oxygen to the cells and releases the sickled red blood cells trapped in the spleen. The spleen reduces in size and the anemia is corrected.

The chances of having another episode of acute splenic sequestration are high. If the first episode was severe or if a second acute splenic sequestration occurs, the doctor may recommend splenectomy (removal of the spleen).

Can a person live without a spleen?

Yes. Other organs in the body perform the same function as the spleen, so a person can have a healthy life without the spleen. However, before a splenectomy, the doctor will make sure all immunizations are up to date, and afterwards, will prescribe daily penicillin to help decrease the risk of infection.

Questions?

Talk to your doctor or nurse case manager if you have more questions about splenic sequestration. Remember, if you suspect acute splenic sequestration, seek medical treatment right away. If in doubt, call:

The Hematology Clinic at 901-595-5041, Monday through Friday 8 a.m.–5 p.m.

After 5 p.m. and on the weekends, call 901-595-3000 and ask to speak to the hematologist on call.


 

This document is not intended to take the place of the care and attention of your personal physician or other professional medical services. Our aim is to promote active participation in your care and treatment by providing information and education. Questions about individual health concerns or specific treatment options should be discussed with your physician.

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