A 30-year-old woman with sickle cell anaemia presents with acute severe pain in her chest, back, and limbs. Temperature is 38.8°C, SpO2 90%, CXR shows new bilateral pulmonary infiltrates, and WBC is 18,000/μL. This is her third episode with a pulmonary infiltrate in 2 years. What is the most critical immediate intervention and long-term disease-modifying therapy?
- A Simple top-up transfusion only; no disease modification needed
- B Exchange transfusion acutely; hydroxyurea for long-term prevention of ACS ✓
- C High-dose steroids acutely; penicillin prophylaxis long-term
- D IV morphine and antibiotics only; no transfusion required
Explanation
Acute chest syndrome (ACS) — new pulmonary infiltrate with respiratory symptoms in sickle cell disease — is a life-threatening emergency. Exchange transfusion (not simple transfusion) is preferred for severe ACS (SpO2 <90%, multi-lobar involvement, rapid deterioration) as it reduces HbS% without increasing total blood viscosity. Hydroxyurea reduces the frequency of ACS, vaso-occlusive crises, and transfusion requirement (MSH trial) and is the cornerstone of long-term disease modification. Steroids are controversial in ACS (rebound crises). Morphine/antibiotics are supportive but not sufficient alone.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.