Sickle Cell Disease and Splenic Sequestration

These instructions from Children's Hospital of Philadelphia are for patients with sickle cell disease to learn more about splenic sequestration.

Important information:

The spleen is a small organ located on the upper left side of the belly. It serves as a filter in the bloodstream to fight infection by removing bacteria from the blood. In patients with sickle cell disease, the spleen does not work as well as it should due to damage caused by sickled red blood cells

Splenic sequestration can happen in patients as young as 2 months old. It happens when a large number of sickle-shaped cells get trapped in the spleen and cause it to suddenly get large. Splenic sequestration is a life-threatening emergency.

Symptoms of splenic sequestration include:

• Rapid heartbeat

• Irritability

• Tiredness or weakness

• Paleness

• Pain or swelling on the left side of the belly

• Fever

• Can be associated with infection

Treatment for splenic sequestration includes:

• Intravenous (IV) fluids

• Blood transfusions

• Splenectomy, the removal of the spleen, if the child has repeated or life-threatening episodes

Patient instructions:

• Check your child's spleen size regularly as recommended

• Call your hematology team immediately if your child has any of the symptoms listed above or their spleen is larger than usual

Please contact your CHOP hematology team with questions or concerns.

Division of Hematology

215-590-3535

Sickle Cell Center

For non-urgent issues, send your team a message in the MyCHOP portal.

Reviewed on June 22, 2023, by Alexandra Kaspin, RN, MSN