Are patients with splenic sequestration crisis typically febrile?

Fever in Splenic Sequestration Crisis

Patients with splenic sequestration crisis typically do not present with fever as a characteristic symptom. 1 While fever may occur in some cases, it is not a defining feature of this condition.

Clinical Presentation of Splenic Sequestration Crisis

• Splenic sequestration crisis 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 these episodes 1
• Severe cases may rapidly progress to shock and death if not promptly recognized and treated 1
• The condition is most common in children with HbSS younger than 5 years and adolescents with HbSC, but can occur at any age in patients with any form of sickle cell disease 1

Associated Symptoms and Presentation

• Abdominal pain, particularly in the left upper quadrant, is a common presenting symptom 2, 3
• Rapid splenic enlargement that can be detected on physical examination 4
• Circulatory compromise may develop as the condition progresses 3
• Low-grade intermittent fever may be present in some cases, but is not a defining characteristic 2
• In a case series of 16 adult patients with ASSC, only four presented with abdominal pain as their primary complaint, while most presented with pain crisis elsewhere in the body 3

Diagnostic Considerations

• Diagnosis is based on clinical findings of an enlarged spleen, decreasing hemoglobin levels, and often thrombocytopenia 4
• The maximum decreases in hemoglobin (up to 42%) and platelets (up to 62%) may not occur until around day 3, potentially delaying identification and treatment 3
• Changes in platelet counts may be more reliable markers than changes in hemoglobin level since red cell transfusions may interfere with assessments of the sequestration process 3

Management Approach

• Prompt recognition and careful administration of red blood cell transfusions are lifesaving interventions 1
• Transfusions of 3 to 5 mg/kg are recommended, 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
• In severe or recurrent cases, surgical splenectomy may be recommended to prevent recurrence 1
• Red cell exchange has been reported as an effective alternative to splenectomy in some cases 5

Prevention and Education

• Parents and caregivers of young children with SCD should be taught to check their child's spleen daily to recognize enlargement early 1
• Urgent medical attention should be sought at the first sign of splenic enlargement 1
• The risk of recurrence is higher when the first episode occurs before 1 year of age compared to after 2 years of age 6

Important Distinctions

• Splenic sequestration should be distinguished from other acute complications of sickle cell disease that more commonly present with fever, such as acute chest syndrome or infection 1
• Fever is a cardinal symptom requiring urgent evaluation in sickle cell patients due to their increased risk of overwhelming infection, but it is not specifically associated with splenic sequestration 1

In summary, while fever may occasionally be present in patients with splenic sequestration crisis, it is not a defining or characteristic feature of this condition. The hallmarks remain rapid splenic enlargement, decreasing hemoglobin, and potential circulatory compromise.