Author: Büsra Yildirim
Co-authors: Hanife Tugba Akcam, Pinar Nalcacioglu Memis
Abstract
Purpose: We aim to draw attention to the atypical lymphoid cell we detected in the vitreous as an early sign of cranial lymphoma in a patient who had radiation retinopathy and previous primary central nervous system lymphoma.Setting/Venue: Ministry of Health Ankara City Hospital, Ankara, Turkey.
Methods: This is a case report of one of our patients in retina clinic.
Results: A 47 year old man presented with a history of vision loss in his left eye for five days. His medical history was significant for primary central nervous system lymphoma (PCNSL) diagnosed one year ago, he states that he received three months of chemotherapy and one month of radiotherapy consisting of 25 sessions. The last session of them was seven months ago and currently he has no medication available. He also reported that two years ago he underwent vitreoretinal surgery on his right eye because of an uveitic reaction but no medical reports available.
On clinical examination, his best-corrected acuities were 20/400 in the right eye and hand motion in the left eye. Anterior segment examination was unremarkable and intraocular pressures were normal and symmetric. Pupils and extraocular motility were unremarkable. Bilateral grade 2 anterior vitreous cells were present; there were a yellowish-whitish coloured retinitis area adjacent to the superotemporal vascular arcade in the right eye, diffuse retinal edema and pale ischemic appearance was seen in the whole retina of left side. Optic disc appearance was normal on both sides. Optic coherence tomography(OCT) findings were both inner and outer retinal edema in retinitis area in right eye, diffuse intraretinal and subretinal fluid accumulation and exudates in left eye.In fundus fluorescein angiography there were bilateral enlargement of foveal avascular zone, fluorescein leakage of parafoveal region and superotemporal retinitis area in right eye, perifoveal leakage and capillary drop out prominent superior in left eye. His preliminary diagnoses were primary intraocular lymphoma, metastasis of the possible residual cranial lymphoma and radiation retinopathy. Vitreous tapping was performed in left eye. Due to insufficient amount of aspirable material, only cytology could be studied from the vitreous sample. Also oncologic examination and cranial-orbital magnetic resonance imaging performed in the following days and no significant finding was detected. One week later his visual acuity increased to counting fingers from a meter in left eye. Cytology resulted as one atypical lymphoid cell in vitreous. In the light of these findings, it was concluded that the patient had radiation retinopathy along with residual vitreoretinal lymphoma. Thereafter, intravitreal anti-VEGF injection was performed. patient was followed up weekly, retinal edema has progressively decreased and visual acuity increased to 20/400 at the and of a month. Also reduction of subretinal and intraretinal fluid observed in OCT images of left. No fundus examination change in right eye was observed. At the end of the first month intravitreal bevacizumab injection was repeated, ten days later there was no subretinal fluid anymore, exudates still present and fundus colour looked a bit normal.
Three months later he had left hemiplegia and amnesia as a result of multiple tumoral lesions in the brain diagnosed by MRI. The patient started receiving chemotherapy again. His visual acuity stayed at 20/400 bilaterally.
Conclusion: The PCNSL is an aggressive -even mortal- variant of extranodal non-Hodgkin lymphoma. An approximate 20% of patients with PCNSL have ocular manifestations including keratouveitis, vitritis, multifocal subretinal infiltrates, sub-RPE deposits, retinal vasculitis, vascular occlusion, exudative retinal detachment, and optic atrophy. Moreover, treatment complications such as radiation retinopathy are sources of strain for both patients and physicians. There is yet be found an optimal treatment algorithm in this disease spectrum where a careful multidisciplinary clinical follow-up is need to make patients’ remaining life more comfortable.
