What is the management of splenic crisis in sickle cell disease (SCD) or sickle beta thalassemia?

Management of Splenic Sequestration Crisis in Sickle Cell Disease and Sickle Beta Thalassemia

Splenic sequestration crisis requires immediate recognition and careful red blood cell transfusion management to prevent progression to shock and death, with consideration for splenectomy after recovery from life-threatening or recurrent episodes. 1

Clinical Presentation and Diagnosis

• Splenic sequestration is characterized by a rapidly enlarging spleen and a decrease in hemoglobin level of more than 2 g/dL below the patient's baseline value 1
• Mild to moderate thrombocytopenia is often present during acute splenic sequestration 1
• Although most common in children with HbSS younger than 5 years and adolescents with HbSC, it may occur at any age in patients with any form of SCD, including adults with sickle cell-hemoglobin C disease or sickle beta thalassemia 1, 2
• Technetium 99m/sulfur colloid scanning during acute splenic sequestration crisis may show almost total lack of splenic uptake or decreased uptake with intrasplenic filling defects 2

Immediate Management

• Prompt recognition and careful administration of red blood cell transfusions are lifesaving interventions 1
• Transfusions of 3 to 5 mg/kg should be used, with post-transfusion hemoglobin checked before the next aliquot of red cells is ordered 1
• Care must be taken 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 event resolves 1
• Aggressive hydration is crucial, with careful monitoring of fluid balance to prevent overhydration 3
• For intravenous hydration, 5% dextrose solution or 5% dextrose in 25% normal saline is recommended rather than normal saline due to hyposthenuria in sickle cell patients 4

Pain Management

• Effective analgesia is crucial if pain is present during splenic sequestration 4
• For severe pain, parenteral opioids such as morphine are indicated and should be administered by scheduled around-the-clock dosing or patient-controlled analgesia 1
• Patient-controlled analgesia (PCA) has been shown to have lower treatment failure rates compared to intermittent injections 5

Monitoring and Supportive Care

• Continuous monitoring of vital signs, oxygen saturation, and hemoglobin levels is essential 3
• Baseline oxygen saturation should be documented, with oxygen therapy administered to maintain SpO2 above baseline or 96% (whichever is higher) 3
• Regular assessment by hematology specialists is recommended for patients with splenic sequestration 3
• Monitor for signs of infection, as infections can precipitate or worsen sickle cell crises 3

Prevention of Complications

• Maintain normothermia, as hypothermia can lead to shivering and peripheral stasis, which increases sickling 1
• Incentive spirometry should be promoted if the patient is hospitalized to prevent acute chest syndrome, especially in patients with thoracoabdominal pain 3
• Thromboprophylaxis should be used for all peri- and post-pubertal patients, and mobilization encouraged when appropriate 1

Long-term Management

• Surgical splenectomy to prevent recurrence may be recommended after recovery from life-threatening or recurrent episodes 1
• Parents and caregivers of young children with SCD should be taught to check their child's spleen daily to recognize enlargement early and urgently seek medical attention 1
• Regular follow-up with hematology specialists is essential for ongoing management and prevention of recurrent episodes 3

Special Considerations

• Splenic sequestration crises can be life-threatening and recurrent, requiring vigilant monitoring and prompt intervention 2
• In adults with sickle cell-hemoglobin C disease or sickle beta thalassemia, splenic sequestration may be underdiagnosed or misdiagnosed as splenic infarctions 2
• Multidisciplinary care involving hematologists, emergency physicians, and potentially surgeons is crucial for optimal management 3

Common Pitfalls and Caveats

• Delayed recognition of splenic sequestration can lead to rapid progression to shock and death 1
• Overtransfusion can lead to complications when sequestered red cells are released from the spleen 1
• Failure to consider splenic sequestration in adults with persistent splenomegaly (particularly those with HbSC or sickle beta thalassemia) may lead to missed diagnosis 2