A 25-year-old woman with sickle cell disease (HbSS) presents with severe bone pain and fever. She is managed with IV fluids, opioid analgesia and antibiotics. Over the next 48 hours she develops progressive hypoxia (SpO₂ 84% on room air), bilateral pulmonary infiltrates and falling Hb from 8.5 to 5.8 g/dL. This complication is best managed with:
- A High-flow oxygen and aggressive IV fluid hydration
- B Exchange transfusion to reduce HbS percentage to <30% and correct hypoxia ✓
- C Hydroxyurea initiation during acute crisis
- D IV methylprednisolone for acute chest syndrome
Explanation
Acute chest syndrome (ACS) — new pulmonary infiltrate with fever/respiratory symptoms — is the leading cause of death in sickle cell disease. For severe ACS with hypoxia (SpO₂ <95% or rapid decline in Hb), exchange transfusion (erythrocytapheresis) to reduce HbS to <30% is the treatment of choice as it reduces viscosity and sickling without the volume overload of simple transfusion. Simple top-up transfusion may be used for mild ACS. Steroids (methylprednisolone) reduce the duration of ACS (as shown in one RCT) but increase the risk of rebound pain crisis and are not recommended routinely. Hydroxyurea prevents future ACS but should not be started during an acute crisis.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.