A 30-year-old man with known sickle cell disease presents with severe acute chest pain, fever, and new bilateral pulmonary infiltrates on CXR. SpO2 is 90% on room air. WBC is 22,000/µL. The diagnosis and most critical acute intervention are:

• A Community-acquired pneumonia — antibiotics alone
• B Pulmonary embolism — anticoagulation with heparin
• C Splenic sequestration — simple top-up transfusion
• D Acute chest syndrome — exchange transfusion plus aggressive supportive care (supplemental oxygen, incentive spirometry, antibiotics) ✓

Explanation

Acute chest syndrome (ACS) in sickle cell disease is defined by new pulmonary infiltrate plus fever and/or respiratory symptoms, and is the leading cause of death in adults with SCD. Management includes supplemental oxygen, incentive spirometry (to prevent atelectasis), broad-spectrum antibiotics (to cover typical and atypical organisms including Chlamydia and Mycoplasma), and simple or exchange transfusion. Exchange transfusion is preferred in severe ACS (SpO2 <90%, rapid deterioration) to reduce HbS percentage below 30% without increasing haematocrit.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.