Medicine · Anemia (Iron Deficiency, Hemolytic, Sickle Cell, Thalassemia)

A 22-year-old man with sickle cell anaemia presents with severe chest pain, fever, tachypnoea, and new bilateral pulmonary infiltrates. SpO₂ is 88% on room air. What is this acute complication and what is its most critical immediate intervention?

  • A Acute chest syndrome; exchange transfusion to reduce HbS below 30%
  • B Pneumonia; IV antibiotics only
  • C Pulmonary embolism; anticoagulation with heparin
  • D Splenic sequestration; simple top-up transfusion
Correct answer: A. Acute chest syndrome; exchange transfusion to reduce HbS below 30%

Explanation

Acute chest syndrome (ACS) — new pulmonary infiltrate with respiratory symptoms (chest pain, fever, hypoxia) in a sickle cell patient — is the leading cause of death in SCD. Severe ACS (SpO₂ <90%, multilobe involvement, rapid deterioration) is an indication for emergency exchange transfusion to rapidly reduce HbS percentage below 30% while maintaining total haemoglobin at 10 g/dL. Simple transfusion may worsen hyperviscosity. Additional management includes oxygen, analgesics, incentive spirometry, empirical antibiotics (including atypicals — Mycoplasma/Chlamydia are common triggers), and bronchodilators.

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

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