A rare case of recurrence presenting with bilateral exudative retinal detachment in a child with acute lymphoblastic leukemia

Abstract

To the Editor: Relapse of leukemia represents the outgrowth of a clonal cell population that could not be eliminated completely by treatment.1 Extramedullary relapse of acute lymphoblastic leukemia (ALL) usually appears in “sanctuary sites”where the tissue–blood barrier is an obstacle to drug delivery.2 Although the ocular involvement of leukemia can be distinguished mainly with ophthalmic examination, difficulties still exist in diagnosis or exclusion. In this regard, advanced ocular imaging may help in analysis of deeper ocular structures, including the retina and choroid, even before significant diseasemanifests. Herein we present a 14-year-old female who was consulted to the ophthalmology department with visual loss in her right eye for the last 2 days. She was diagnosed with pre-B-ALL 9 months ago and no extramedullary involvement was present at that time. Ocular fundus examination was normal and her lumbar puncture (LP) contained no cells. She was stratified into high-risk group based on minimal residual disease (MRD) study results according to theALL IC-BFM-20093.3 She was considered to be a candidate for hematopoietic stem cell transplantation (HSCT) in first complete remission (CR1) with 8% blasts and high MRD result (four markers: 10–3) on day 33 bone marrow (BM). Complete hematologic remission and MRD negativity were achieved after completing three high-risk blocks of chemotherapy. Nine months later, the patient developed visual loss in her right eye during pretransplant screening tests. Ophthalmic examination showed that best corrected visual acuity (BCVA) was 20/200 in the right eye and 20/20 in the left eye. Examination of cornea, anterior chamber, lens, and optic discs were normal bilaterally. There were no evidences of papilledema and retinal hemorrhage in both eyes; however, serous elevation of the retina andmacula were present in the right eye (Figure 1A). Exudative retinal detachment was confirmed by significantly elevated subretinal fluid on optical coherence tomography (OCT) of the right eye (Figure S1A). Choroidal thickness measurements, retinal imaging results, and funduscopywerewithin normal limits for the left eye (Figures 1B and S1B). In the following days, exudative retinal detachment remained in the right eye (Figures S1C and S2A). The choroid of the left eye got thickened and exudative retinal detachment developed this time in the fellow eye (Figures S1D and S2B). BCVA was 20/40 in the right eye and 20/100 in the left eye then. Fluorescein angiography of retinal vasculature showed characteristic pin-point leaks in the posterior pole of both eyes (Figure1C,D). Thepossibility of ocular diseases includingHarada’s F IGURE 1 (A) Exudative retinal detachment in the right eye at presentation. (B) Fundus photography of the left eye at presentation. Please note that themacula is quite normal. (C) Fundus fluorescein angiography of the right eye showing characteristic hyperreflective pin-point leaks in themacular of the right eye and around the optic nerve. (D) Fundus fluorescein angiography of the right eye showing characteristic hyperreflective pin-point leaks around the optic nerve of the left eye