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Differentiation Syndrome (ATRA Syndrome)

Imaging findings

Serial chest radiographs in a patient undergoing all-trans-retinoic acid (ATRA) therapy for acute promyelocytic leukemia (APL) demonstrate rapid onset of diffuse interstitial and alveolar opacities, cardiomegaly, and bilateral pleural effusions, which resolve quickly following administration of dexamethasone.

Key takeaways

Differentiation syndrome (formerly ATRA syndrome) is a life-threatening complication of APL therapy with differentiating agents (ATRA or arsenic trioxide). It is caused by the rapid maturation and infiltration of promyelocytes, leading to cytokine release and capillary leak. Typical findings include dyspnea, fever, weight gain, pulmonary edema, and pleural effusions. Treatment requires prompt initiation of high-dose corticosteroids.

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