Successful treatment of retinoic acid syndrome with high-dose dexamethasone pulse therapy in a child with acute promyelocytic leukemia treated with ATRA

A 5 year old female developed femoral pain, fever, and hemorrhagic tendency. She was diagnosed as having acute promyelocytic leukemia (APL). Approximately 2 weeks after the administration of all-trans retinoic acid (ATRA), she developed a high fever, edema, and respiratory distress which met the criteria for retinoic acid syndrome. At first, we tried to treat the patient with oral corticosteroid, however, this approach was unsuccessful. Considering the worsening of her condition, we then chose to administer a large dose of intravenous dexamethasone therapy for 3 days. Immediately after this therapy, she became afebrile, respiratory distress and edema disappeared, and there was a general improvement of the symptoms. All-trans retinoic acid at the reduced dose of 25 mg/m², was continued for an additional 6 weeks and then discontinued. Since the cessation of dexamethasone and ATRA, there has been no relapse of APL in this patient. Although based on only one case, we recommend the intravenous high-dose dexamethasone pulse therapy (13 mg/m² per day, for 3 days) for treating retinoic acid syndrome which develops in pediatric APL patients treated with ATRA.