An 18-month-old boy with HbSS suddenly becomes lethargic and pale. His usual hemoglobin of 8 g/dL has fallen to 3.4 g/dL, platelets are low normal, and reticulocytes are 2%. The spleen is palpable 6 cm below the costal margin, larger than documented previously. Blood pressure is 82/50 mmHg with pulse 160 per minute. What is the immediate priority?

• A Emergency splenectomy
• B Urgent red cell transfusion ✓
• C Bone marrow examination
• D Intravenous immunoglobulin infusion

Explanation

Acute splenic sequestration occurs when sickled cells trap in the enlarging spleen, causing rapid hemodynamic collapse with a hemoglobin drop of 2 g/dL or more and falling platelets, typical before age 5. Immediate volume resuscitation and red cell transfusion is life saving. Splenectomy is considered later for recurrent episodes, not during the acute event. The reticulocyte count remains adequate here, distinguishing this from aplastic crisis caused by parvovirus A19, where marrow failure gives reticulocytopenia without splenomegaly.

Reference: Nelson Textbook of Pediatrics, 22nd ed.

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Written and medically reviewed by the StethoPrep medical team.