Retinoic Acid Syndrome: A Case Report and Review

Background: The treatment of acute promyelocytic leukemia with all-trans-retinoic acid (ATRA) sometimes results in a syndrome characterized by fever, respiratory distress, weight gain, pleural or pericardial effusion, and pulmonary infiltrates. We report the major clinical and radiologic features of ATRA syndrome. Materials and Methods: In the past, occasional case reports and literature pertaining to ATRA syndrome. The purpose of our report and the literature review is to heighten physicians awareness of this syndrome, which often manifests as nonspecific clinical and radiographic findings. Results: The etiopathogenesis of the syndrome remain unclear. The Incidence of the syndrome has varied in reports from 5% to 27% and the mortality from 5%-29%. The time of time onset of ATRAS varies. The reported median time to the occurrence of ATRA syndrome is 7-12 days. Chest radiographs show increased cardiothoracic ratio in 64% of the patients, increased vascular pedicle width in 76%, increased pulmonary blood volume in 82%, ground-glass opacity in 57%, consolidation in 60%, nodules in 60% of the patients. Pleural effusion is noted in 75% of the patients either unilateral or bilateral effusions. Pulmonary hemorrhage is developed in about 20% of the patients during course of ATRAS. Conclusions: Diagnosis of ATRAS manifestations and immediately starting corticosteroids after the diagnosis of ATRAS may be improve the patients outcome. Because the radiologic features of ATRAS are nonspecific, it would be impossible to differentiate one from the other based solely on these features. . Prompt administration of steroids is critical, not only when the diagnosis is definitively established, but also at the first sign of unexplained dyspnea, fever, weight gain, or pulmonary infiltrates.