Sickle Cell Aplastic Crisis

Acute splenic sequestration is sudden trapping of a large volume of blood within an enlarged spleen, causing rapid haemoglobin fall and potentially fatal hypovolaemic shock.

It occurs predominantly in infants and young children with sickle cell disease before autosplenectomy develops.

• Sudden weakness or lethargy
• Marked pallor
• Rapidly enlarging spleen
• Tachycardia
• Abdominal distension or pain
• Shock

• Compare haemoglobin with baseline. A fall of at least approximately 2 g/dL with acute splenomegaly strongly supports sequestration.

• Reticulocytosis is usually present, distinguishing sequestration from aplastic crisis.

• Crossmatch blood immediately.

• Stabilize circulation.

• Give packed red cells approximately 5 to 10 mL/kg, reassessing carefully because sequestered blood can re enter the circulation as the spleen contracts, increasing the risk of hyperviscosity if overtransfused.

• Avoid raising haemoglobin excessively above the patient's usual level.

• Teach caregivers how to palpate the spleen.

• Recurrent episodes require haematology assessment for chronic transfusion strategy or splenectomy depending on age and disease pattern.