Retinoic acid syndrome after one dose of all-transretinoic acid

Abstract

All-trans-retinoic acid (ATRA) is typically used as a first line agent in the treatment of acute promyelocytic leukaemia (APL), achieving complete remission (CR) rates (incombination with chemotherapy) of about 90%. One of the drawbacks of the use of ATRA is that up to 30% of patients can present with retinoic acid syndrome (RAS), which can be fatal in some patients. We describe a case of RAS after only one dose of ATRA, which to our knowledge has not previously been identified in the literature. The pathophysiology and treatment of APL is presented. A clinical description of RAS is outlined, and an evaluation of risk factors for developing RAS is reviewed.