Differentiation Syndrome with Ocular Manifestations in Acute Promyelocytic Leukemia Patients Treated with All-trans Retinoic Acid

Purpose: To report a rare case of differentiation syndrome with ocular manifestations in a patient with acute promyelocytic leukemia treated with all-trans retinoic acid (ATRA). Case summary: A 27-year-old female complained of yellowing of vision and decreased visual acuity during an ATRA medication course for acute promyelocytic leukemia. Bilateral diffuse drusen-like lesions were found at the posterior pole along with multiple pigment epithelial detachment on optical coherence tomography (OCT) scans. Fluorescein angiography showed multiple hyperfluorescent lesions with leakage at the late phase corresponding to drusen-like lesions in fundus photography. Indocyanine green angiography revealed multiple hypocyanescent lesions. ATRA treatment was discontinued and replaced with high-dose dexamethasone. Accordingly, the patient experienced a rapid improvement in visual symptoms and the chorioretinal lesions on OCT scans showed marked resolution. Conclusions: Differentiation syndrome-associated chorioretinopathy may occur in patients with acute promyelocytic leukemia treated with ATRA. Because the occurrence of chorioretinopathy may be associated with systemic aggravation of the ATRA syndrome, preemptive treatment with early detection is required.