Splenic sequestration is a serious complication of sickle cell disease that happens when blood is trapped in the spleen. This causes the spleen to enlarge quickly. Splenic sequestration may also be called spleen crisis, acute spleen enlargement, or acute splenic sequestration.
Splenic sequestration is more common in infants and young children but can happen at any age. Treatment may include a red blood cell transfusion, IV fluids, or other supportive care.
Splenic sequestration is a medical emergency and can be life-threatening. If your child has signs or symptoms of splenic sequestration, contact your care team or go to the emergency room right away.
In splenic sequestration, blood enters the spleen and is trapped by sickled red blood cells, causing the spleen to enlarge quickly.
How the spleen works
The spleen works by filtering blood to help the immune system protect the body from infection. It is an organ in the upper left side of the abdomen (belly). It is about the size of a fist.
The spleen helps:
Remove old or damaged blood cells and other waste from the blood
Store blood
Fight infection
Symptoms of splenic sequestration
Splenic sequestration symptoms can develop suddenly. Watch for signs and symptoms, especially if your child’s spleen becomes larger than usual. Your care team will show you how to feel your child’s spleen so you know its normal size.
Signs and symptoms of splenic sequestration include:
A spleen that suddenly becomes larger, firmer, or easier to feel
Pain or fullness in the upper left side of the abdomen
Pale skin, lips, or gums
Weakness, fatigue, unusual sleepiness, or trouble waking up
If your child has trouble breathing, becomes very pale, weak, or limp, or is not responding, seek emergency medical help right away.
Causes of splenic sequestration
Splenic sequestration happens when sickled red blood cells get trapped in the spleen. This causes the spleen to fill with blood and become enlarged.
The blood in the spleen becomes separated (sequestered) from the blood in the body. When blood is trapped in the spleen, less blood moves through the rest of the body. This can cause a severe drop in red blood cells (anemia) and lower circulating blood volume. If it is not treated quickly, the body can go into shock.
Diagnosis of splenic sequestration
Splenic sequestration is diagnosed based on your child’s medical history, physical exam, and blood tests. During the physical exam, your care team will check whether your child’s spleen is enlarged. Tests may include:
The number of red blood cells, white blood cells, and platelets
Hematocrit, the percentage of blood made up of red blood cells
Hemoglobin, the protein in red blood cells that carries oxygen
A CBC can show a sudden drop in hemoglobin, hematocrit, and platelets compared with your child's usual levels, which is a sign of splenic sequestration. Platelets, like red blood cells, can be trapped in the spleen during sequestration.
Reticulocyte count to measure the number of young red blood cells in your child’s blood. A high reticulocyte count may mean that the bone marrow is making more red blood cells to replace those trapped in the spleen.
Ultrasoundof the spleen may be used if it cannot be felt easily during the physical exam or if the diagnosis is uncertain.
Treatment of splenic sequestration
Splenic sequestration is a medical emergency. Treatment depends on your child’s symptoms and how severe the condition is.
Care in the hospital
Your child will be admitted to the hospital if your care team suspects splenic sequestration. This allows for close monitoring and treatments based on your child’s needs.
IV fluids and supportive care
IV (intravenous) fluids are given to help with hydration or blood flow. Your child may also need oxygen, antibiotics, or other treatments depending on symptoms or if an infection is suspected.
Blood transfusion
A red blood cell transfusion may be needed to help restore red blood cells and blood volume. As blood flow improves, the spleen often gets smaller and symptoms improve.
Splenectomy
If your child has splenic sequestration, the risk of getting it again is high. Your care team may recommend surgery to remove the spleen (splenectomy) if your child has severe or repeated episodes of splenic sequestration.
Living without a working spleen
Your child can live a healthy life without a working spleen. This may happen if the spleen has been removed (splenectomy) or if repeated damage from sickle cell disease causes it to stop working over time (autosplenectomy).
Some people with sickle cell disease may have a spleen that stays enlarged over time. This is called chronic splenomegaly and may not cause symptoms. It is more common in some types of sickle cell disease, for example, those with HbSC disease and HbSβ⁺ thalassemia.
Because the spleen is part of the immune system, your child has a higher risk of infection if the spleen does not work properly. Your care team may recommend vaccines and preventive antibiotics to reduce this risk.
When to contact your care team
Splenic sequestration is a medical emergency. If your child’s spleen is suddenly larger than normal or your child has other signs of splenic sequestration, contact your care team or go to the emergency room immediately.
Call emergency medical services (911 in the U.S.) if your child:
Has trouble breathing
Becomes very pale, weak, or limp
Is hard to wake up
Is confused or not responding
Living with splenic sequestration
If your child has had splenic sequestration, it is important to watch for symptoms of another episode. Keep all medical appointments so your care team can monitor your child’s sickle cell disease and overall health.
Your care team might recommend a medical ID bracelet for your child. They may also advise avoiding certain high-contact sports or activities that could injure the spleen.
Questions to ask your care team
Is my child at higher risk for splenic sequestration?
What symptoms of spleen crisis should I watch for?
How do I check my child’s spleen for changes in size?
How often should I check my child’s spleen?
What should I do if I think my child’s spleen feels larger?
What are the possible complications of splenic sequestration?
What treatment does my child need?
What emergency warning signs should I watch for?
When should I call the care team, go to the emergency room, or call 911?
Key points about splenic sequestration
Splenic sequestration (spleen crisis) happens when blood gets trapped in the spleen, causing it to enlarge quickly.
Splenic sequestration is a medical emergency that can lead to severe anemia, shock, and even death if it is not treated promptly.
A spleen that suddenly becomes larger than usual is an important warning sign.
Treatment for splenic sequestration often includes a red blood cell transfusion.
Your child may also need IV fluids, oxygen, antibiotics, or other supportive care.
Some children who have severe or repeated episodes may need surgery to remove the spleen (splenectomy).
Learning how to check your child’s spleen and recognize symptoms early can help you get treatment quickly.
Akshay Sharma, bone marrow transplant physician at St. Jude, discusses things people may not know about sickle cell disease. Learn more about sickle cell.
