A 22-year-old man with sickle cell disease presents with severe chest pain, fever, tachycardia, and worsening hypoxia with new pulmonary infiltrate on CXR. His SpO2 has dropped from 98% to 88% over 12 hours. What is this complication and the most important specific treatment?
- A Acute chest syndrome; simple or exchange blood transfusion ✓
- B Pulmonary embolism; anticoagulation with heparin
- C Pneumococcal pneumonia; IV penicillin alone
- D Fat embolism; supportive care only
Explanation
Acute chest syndrome (ACS) is the leading cause of death in sickle cell disease, defined by new pulmonary infiltrate plus at least one of: fever, chest pain, or respiratory symptoms. Pathophysiology involves sickling in pulmonary vasculature causing in-situ thrombosis, fat embolism from infarcted marrow, and infection. The key specific treatment is blood transfusion — simple transfusion to achieve Hb 10 g/dL or exchange transfusion (if Hb >10 or rapidly deteriorating) to dilute HbS below 30%. Supplemental O2, hydration, incentive spirometry, analgesia, and empiric antibiotics (ceftriaxone + azithromycin) are also given.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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