A 22-year-old man with sickle cell disease (HbSS) presents with acute severe chest pain, fever 38.8°C, and oxygen saturation 91% on room air. CXR shows new bilateral lower zone infiltrates. This is acute chest syndrome (ACS). In addition to oxygen, analgesics, and antibiotics, which intervention has the most immediate impact on severity if the patient continues to deteriorate?
- A Hydroxyurea initiation
- B Exchange transfusion to reduce HbS to <30% ✓
- C Deferoxamine chelation
- D High-dose corticosteroids
Correct answer: B. Exchange transfusion to reduce HbS to <30%
Explanation
Exchange transfusion (erythrocytapheresis) is the most effective acute intervention for severe or deteriorating acute chest syndrome; it rapidly reduces HbS percentage below 30%, improving oxygen delivery and reversing vaso-occlusion and haemolysis without the volume overload of simple transfusion. Simple top-up transfusion may be used for mild cases. Hydroxyurea is for long-term prevention. Corticosteroids are controversial and may precipitate rebound vaso-occlusion.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.