A 35-year-old African-American man presents with severe back and chest pain after a long-haul flight. His known sickle cell disease is usually well-controlled. Hemoglobin drops from 8.5 to 6.2 g/dL. LDH rises to 980 U/L, indirect bilirubin increases, and he develops oxygen saturation of 88%. CXR shows new bilateral infiltrates. The diagnosis and immediate intervention priority is:
- A Acute chest syndrome — exchange transfusion, broad-spectrum antibiotics, oxygen, incentive spirometry ✓
- B Pneumonia — start antibiotics only
- C Pulmonary embolism — anticoagulation with heparin
- D Simple vaso-occlusive crisis — analgesics and IV fluids only
Correct answer: A. Acute chest syndrome — exchange transfusion, broad-spectrum antibiotics, oxygen, incentive spirometry
Explanation
Acute chest syndrome (ACS) in sickle cell disease is defined by new pulmonary infiltrate + chest pain + fever + hypoxia, and carries a mortality of 1–3% per episode. Management requires oxygen supplementation, empirical antibiotics (covering atypical organisms — azithromycin + cephalosporin), analgesics, incentive spirometry (critical to prevent atelectasis), and exchange transfusion to reduce HbS <30% when rapidly worsening. Simple transfusion can worsen hyperviscosity; exchange is preferred in severe ACS. Anticoagulation is not routinely indicated.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.