Retinoic Acid Syndrome and Pseudotumor Cerebri in Patients with Acute Promyelocytic Leukemia

ABSTRACT

PURPOSE: To report cases of a retinal, preretinal and vitreous hemorrhage due to retinoic acid syndrome and a papilledema caused by increased intracranial pressure in patients with acute promyelocytic leukemia treated with all-trans retinoic acid (ATRA). CASE SUMMARY: (Case 1) A 48-year-old female visited our clinic with headache, dyspnea, and visual disturbance during ATRA medications for acute promyelocytic leukemia. Her visual acuity of both eyes was hand motion, and fundus examination revealed extensive retinal, preretinal, and vitreous hemorrhage. The laboratory test showed leukocytosis and computed tomography of brain and chest revealed subarachnoid hemorrhage and pulmonary alveolar hemorrhage. She was diagnosed with retinoic acid syndrome and was treated with vitrectomy in both eyes. (Case 2) A 17-year-old male, who had been treated with ATRA for acute promyelocytic leukemia, had headache and visual disturbance. His best-corrected visual acuity was 1.0 in both eyes, but the fundus examination showed papilledema and retinal hemorrhage in both eyes. Goldmann visual field examination revealed an enlarged blind spot in both eyes. He was diagnosed with papilledema caused by ATRA induced increased intracranial pressure, and was observed. CONCLUSIONS: Patients with acute promyelocytic leukemia treated with ATRA may have retinoic acid syndrome and increased intracranial pressure that could result in retinal hemorrhage, vitreous hemorrhage, and papilledema.