A 30-year-old woman with sickle cell disease presents with acute severe chest syndrome — fever, pleuritic chest pain, new pulmonary infiltrate, and SpO2 88%. What is the MOST appropriate treatment?
- A Simple top-up transfusion to Hb > 12 g/dL
- B Hydroxyurea started immediately
- C Exchange transfusion (erythrocytapheresis) to reduce HbS below 30% ✓
- D High-dose systemic corticosteroids
Correct answer: C. Exchange transfusion (erythrocytapheresis) to reduce HbS below 30%
Explanation
Acute chest syndrome (ACS) is a leading cause of death in sickle cell disease. Severe ACS (SpO2 <90%, rapidly deteriorating) requires exchange transfusion (erythrocytapheresis) to rapidly reduce the percentage of HbS to <30% while maintaining normal total Hb, avoiding the hyperviscosity risk of simple transfusion. Simple transfusion to high Hb levels worsens viscosity. Hydroxyurea is for long-term prophylaxis, not acute crisis. Corticosteroids may worsen rebound sickling and are not standard for ACS.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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