From the Guidelines
Splenic sequestration is an acute illness characterized by a rapidly enlarging spleen and a decrease in hemoglobin level of more than 2 g/dL below the patient’s baseline value, often accompanied by mild to moderate thrombocytopenia. This condition is a serious complication primarily affecting individuals with sickle cell disease, where large amounts of blood become trapped within the spleen, causing it to rapidly enlarge 1. The hallmarks of splenic sequestration include sudden splenic enlargement, a rapid drop in hemoglobin levels, and symptoms of hypovolemic shock such as weakness, pale skin, rapid breathing, increased heart rate, and abdominal pain.
Key Characteristics
- Rapidly enlarging spleen
- Decrease in hemoglobin level of more than 2 g/dL below the patient’s baseline value
- Mild to moderate thrombocytopenia
- Symptoms of hypovolemic shock
Treatment and Prevention
Treatment of splenic sequestration involves prompt recognition and careful administration of red blood cell transfusions, when appropriate, to restore blood volume and hemoglobin levels 1. It is crucial to avoid acute overtransfusion to a hemoglobin greater than 10 g/dL, as sequestered red cells may be acutely released from the spleen as the acute event resolves. For patients who experience recurrent episodes, preventive measures may include regular transfusions or splenectomy (surgical removal of the spleen) 1.
Important Considerations
- Parents of children with sickle cell disease should be taught how to palpate the spleen to detect enlargement early, as prompt recognition and treatment significantly improve outcomes in this potentially life-threatening condition 1.
- Splenic sequestration is most common in children with sickle cell disease between 6 months and 5 years of age, before the spleen becomes damaged and scarred from repeated sickling episodes.
From the Research
Definition of Splenic Sequestration
- Splenic sequestration refers to a complication of sickle cell disease, where red blood cells become trapped in the spleen, leading to a range of symptoms and potentially life-threatening consequences 2, 3, 4, 5.
- Acute splenic sequestration crises are characterized by progressive splenic enlargement, transfusion-dependent anemia, and circulatory compromise 6.
- The condition can lead to high mortality rates and frequent recurrence in survivors of first attacks, making it a significant concern for individuals with sickle cell disease 2, 3.
Key Features of Splenic Sequestration
- Recurrent acute splenic sequestration crisis is a common indication for splenectomy in patients with sickle cell disease 4.
- Splenectomy, either total or partial, can prevent further sequestration or reduce the recurrence of acute splenic sequestration crises 2, 3.
- However, there is a lack of evidence from trials showing that splenectomy improves survival and decreases morbidity in people with sickle cell disease 2, 3.
Management and Complications
- Splenectomy is a common treatment for splenic sequestration, but it carries its own risks and complications, such as infection and thrombosis 4.
- Red cell exchange is an alternative treatment that can alleviate symptoms and avoid splenectomy complications, especially in young patients 6.
- Abdominal ultrasound should be done routinely preoperatively for all children with sickle cell disease undergoing splenectomy to identify asymptomatic gallstones 4.