This review synthesizes current knowledge on differentiation syndrome (DS) in acute promyelocytic leukemia (APL), covering pathogenesis (PML::RARA degradation, cytokine storm, endothelial injury), diagnostic criteria, severity grading, risk stratification, and treatment strategies including prophylactic, therapeutic, and refractory-phase approaches.
The review proposes a graded, risk-adapted treatment framework for DS management, with emphasis on special populations, predictive models, and emerging therapies, aimed at reducing DS-associated morbidity and mortality in this highly curable disease.
As a narrative review, it does not present original trial data or meta-analytic pooled estimates; specific effect sizes, event rates, or comparative outcomes are not reported. Recommendations may reflect expert consensus rather than high-level prospective evidence.
Clinicians managing APL on ATRA and/or arsenic trioxide should apply a stepwise, risk-adapted approach to DS — from prophylaxis through refractory-phase rescue — using standardized diagnostic criteria and severity grading to guide timely intervention.
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