What Is Splenic Sequestration in Sickle Cell Disease?

Splenic sequestration is a potentially life-threatening condition that’s most commonly seen as a complication of sickle cell disease (SCD). It happens mostly in children. The condition causes your child’s spleen to get bigger and lowers the amount of oxygen-carrying red blood cells in her body.

What Happens in Splenetic Sequestration?

Sickle cell disease affects how your child’s body makes hemoglobin. It’s an important part of her red blood cells, which carry oxygen to the rest of her body. These are usually flat, flexible disks. With sickle cell disease, they become stiff and shaped like a crescent, or a sickle. They don’t live as long as ordinary red blood cells and sometimes get stuck in her blood vessels.

The spleen is an organ in the upper left part of your child’s belly. It helps her body fight infection by cleaning germs out of her bloodstream. But sickle-shaped cells can block blood flow through the spleen. It fills with blood and can become swollen and painful. Doctors call that splenic sequestration.

Because she doesn’t have enough blood cells to carry oxygen to the rest of her body, she could also get severe anemia. It’ll make her feel weak and tired.

How Common Is It?

About 100,000 people in the U.S. have sickle cell disease. About one out of every 365 African-American babies is born with this illness. It happens less often in people from Latin America, the Caribbean, the Middle East, and South Asia.

Splenetic sequestration affects about 30% of children who have SCD, but you can get it at any age, with any form of SCD.

What Are the Symptoms?

Pain on the left side of your child’s belly is the most common. Others include:

Learn to feel your child’s spleen and call a doctor if it gets bigger. Splenic sequestration can cause a dangerous drop in red blood cells even if other symptoms aren’t showing up yet. She’ll need treatment in a hospital.

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Diagnosis

The doctor will:

  • Check for a swollen spleen
  • Test your child’s blood to look for a lower number of red blood cells or other material that makes up her blood, like white blood cells or platelets
  • Use a test like an X-ray or CT scan to take a picture of her insides

How Is It Treated?

A blood transfusion is the usual method. If a lot of blood is trapped in her spleen, the doctor may have to remove some during this process.

If your child’s had splenic sequestration once, she’s likely to get it again, perhaps several times. After several events, doctors might suggest removing her spleen to prevent another attack.

WebMD Medical Reference Reviewed by Laura J. Martin, MD on February 15, 2018

Sources

SOURCES:

CDC: “Sickle Cell Disease (SCD): Complications and Treatments,” “Sickle Cell Disease (SCD): Data & Statistics.”

Klinische Padiatrie: “Splenic sequestration in patients with sickle cell disease.”

Nemours Children’s Health System: “Sickle Cell Disease.”

National Heart, Lung, and Blood Institute: “Sickle Cell Disease.”

New England Pediatric Sickle Cell Consortium: “Splenic Sequestration in Sickle Cell Disease.”

UpToDate: “Overview of the clinical manifestations of sickle cell disease.”

American Family Physician: “Sickle Cell Disease in Childhood: Part II. Diagnosis and Treatment of Major Complications and Recent Advances in Treatment.”

Clinical Pediatric Emergency Medicine: “Sickle Cell Disease in the Emergency Department: Atypical Complications and Management.”

Pediatric Radiology: “CT imaging of splenic sequestration in sickle cell disease.”

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