A 28-year-old man with sickle cell disease presents with severe acute chest syndrome (bilateral infiltrates, oxygen saturation 88% on room air, fever, and chest pain). His haemoglobin is 6.2 g/dL and WBC 22,000. Which is the most urgent disease-specific intervention?
- A Simple or exchange blood transfusion to raise HbS < 30% ✓
- B Hydroxyurea 15 mg/kg started immediately
- C IV deferoxamine for acute iron chelation
- D Bronchial artery embolisation
Correct answer: A. Simple or exchange blood transfusion to raise HbS < 30%
Explanation
Acute chest syndrome (ACS) is a leading cause of death in SCD. Severe ACS (hypoxia, bilateral infiltrates, multi-lobar involvement) requires urgent exchange transfusion to reduce HbS percentage to < 30% while maintaining total haemoglobin around 10 g/dL. Exchange transfusion is preferred over simple transfusion in severe ACS to avoid hyperviscosity from raising haemoglobin rapidly. Hydroxyurea reduces frequency of ACS long-term but has no role in acute management. Iron chelation and bronchial embolisation are not ACS treatments.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.