A 28-year-old man with acute promyelocytic leukaemia started on all-trans retinoic acid two days ago develops fever, dyspnoea, hypoxia, bilateral pulmonary infiltrates, pleural effusion, and weight gain. He is haemodynamically stable. What is the most appropriate immediate management?
- A Stop all-trans retinoic acid permanently and switch to arsenic trioxide alone
- B Give dexamethasone, temporarily withhold all-trans retinoic acid, and provide supportive care ✓
- C Start broad-spectrum antibiotics and continue all-trans retinoic acid unchanged
- D Give furosemide alone and continue all-trans retinoic acid unchanged
Explanation
Differentiation syndrome results from maturing promyelocytes releasing cytokines and adhesion molecules causing capillary leak, typically within days to weeks of starting all-trans retinoic acid or arsenic trioxide. Standard management is immediate dexamethasone with temporary discontinuation of the differentiating agent until resolution, then cautious reintroduction. Antibiotics alone miss the steroid-responsive process, and diuretics alone do not address the underlying inflammatory leak. Permanent cessation is unnecessary because most patients tolerate reintroduction after steroids control the episode.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.