An 11-month-old boy with known HbSS disease suddenly becomes lethargic and pale. His spleen is palpable 8 cm below the costal margin, larger than documented previously. Hb has fallen from 8.5 to 3.9 g/dL, platelets are 90,000/mm³, and reticulocytes are 1.2%. The immediate priority is:
- A Urgent packed red cell transfusion ✓
- B Exchange transfusion
- C Hydroxyurea initiation
- D Observation with serial haemoglobin only
Correct answer: A. Urgent packed red cell transfusion
Explanation
Acute splenic sequestration is defined by acute splenomegaly, a fall in haemoglobin of at least 2 g/dL from baseline, and a falling platelet count, driven by sudden trapping of blood in the spleen. It is a leading cause of death in infants with sickle cell anaemia and demands urgent simple transfusion. Exchange transfusion is reserved for stroke or severe acute chest syndrome. Hydroxyurea addresses long-term frequency, and observation alone risks cardiovascular collapse.
Reference: Nelson Textbook of Pediatrics, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.