Published: 18 Jun 2025

ICD9: 289.52      ICD10: D57.02      ICD11: 3A51.2

Splenic sequestration is a medical emergency that occurs primarily in individuals with sickle cell disease (SCD).
It involves the sudden and significant pooling of a large amount of blood in the spleen.

Here's a breakdown of what it is and why it's dangerous:

What happens: The spleen, which normally filters blood, becomes enlarged as it traps red blood cells, platelets, and other blood components. This trapping is particularly pronounced with the abnormal sickle-shaped red blood cells in SCD.

Why it's dangerous:
Severe Anemia: The most immediate danger is a rapid and drastic drop in hemoglobin levels, leading to severe anemia. This is because a large portion of the circulating red blood cells are trapped in the spleen and unavailable to carry oxygen throughout the body.
Hypovolemia (low blood volume): The pooling of blood in the spleen reduces the amount of blood circulating in the rest of the body. This leads to hypovolemia, which can cause shock and organ damage.
Splenomegaly (Enlarged Spleen): The spleen becomes significantly enlarged, causing abdominal discomfort and sometimes pain.
Thrombocytopenia (low platelet count): Platelets can also be trapped in the spleen, leading to a decrease in platelet count, which increases the risk of bleeding.

Who is at risk: Primarily affects children with sickle cell disease, especially those under the age of 5. However, it can occur in older children and adults with SCD as well. It's less common in sickle cell trait.

Symptoms:
Sudden weakness or fatigue
Pale skin
Rapid heartbeat
Abdominal pain or swelling (due to the enlarged spleen)
Dizziness or lightheadedness
Irritability (in children)

Diagnosis: Diagnosis is typically made based on the sudden drop in hemoglobin levels, physical examination showing an enlarged spleen, and ruling out other causes of anemia.

Treatment:
Blood Transfusion: The primary treatment is blood transfusion to replace the lost red blood cells and correct the anemia.
Intravenous Fluids: IV fluids are given to address hypovolemia and improve circulation.
Monitoring: Close monitoring of vital signs, hemoglobin levels, and spleen size is essential.
Splenectomy (Spleen Removal): In cases of recurrent splenic sequestration, splenectomy (surgical removal of the spleen) may be recommended to prevent future episodes. This is usually considered when other measures have failed and the risk of repeated sequestration outweighs the risks associated with spleen removal. After splenectomy, patients need to be vaccinated and take prophylactic antibiotics due to increased susceptibility to infections.

Prevention:
Regular monitoring of hemoglobin levels, especially in children with SCD.
Prompt medical attention for any symptoms suggestive of splenic sequestration.
Hydroxyurea: This medication can help prevent vaso-occlusive crises in people with SCD, which can indirectly reduce the risk of splenic sequestration.
Transfusions: Regular chronic transfusions can reduce the risk

Important Considerations:

Splenic sequestration is a life-threatening emergency that requires prompt medical intervention. Delay in treatment can result in death.
Parents and caregivers of children with sickle cell disease need to be educated about the signs and symptoms of splenic sequestration and the importance of seeking immediate medical attention if they suspect it.

In summary, splenic sequestration is a serious complication of sickle cell disease involving the rapid pooling of blood in the spleen, leading to severe anemia, hypovolemia, and potentially life-threatening complications. Prompt recognition and treatment are crucial for survival.