A Race Against Time: Early-Onset Differentiation Syndrome Following All-Trans-Retinoic-Acid (ATRA) Therapy in Acute Promyelocytic Leukemia (AML-M3)

Abstract

This study reports a case of differentiation syndrome, a rare complication of ATRA (all-trans-retinoic-acid) therapy, observed in a 20-year-old male with acute promyelocytic leukemia (APML). Following the initiation of ATRA therapy for APML, the patient presented with fever, bleeding gums, bloody stool, and mouth ulcers. After 36 hours, he developed respiratory distress, hypotension, tachycardia, and hypoxemia, leading to the diagnosis of differentiation syndrome. ATRA therapy was promptly discontinued, and the patient, exhibiting type 1 respiratory failure, necessitated intubation. The management included hydroxyurea, dexamethasone, vasopressors, intravenous fluids, and furosemide. After seven days, significant improvement was observed, underscoring the importance of recognizing and promptly addressing differentiation syndrome in APML patients undergoing ATRA therapy. This case emphasizes the necessity of ATRA discontinuation, coupled with the judicious use of steroids and hydroxyurea, in the effective management of differentiation syndrome.