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Unilateral retinopathy secondary to occult primary intraocular lymphoma

  • Clinical Case Report
  • Published:
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Abstract

Purpose

The purpose of the study is to report the clinical case of a 53-year-old woman whose presenting manifestation of primary intraocular lymphoma (PIOL) was unilateral retinal degeneration.

Method

A case report was created with review of clinical, imaging, electrophysiologic, and pathological investigations.

Results

A 53-year-old woman with a distant history of ocular herpes simplex developed progressive central visual loss and intermittent photopsia over 4 years in her right eye. Ophthalmic examination revealed reduced visual acuity OD, central scotoma, and minimal ocular findings. Autofluorescence and infrared imaging revealed mild reflectance changes in the temporal macula, and spectral-domain optical coherence tomography identified mild disruptions of inner segment/outer segment junctions in the subfoveal region of the right eye. A mild window defect was seen on fluorescein angiography. Electrophysiology with multifocal electroretinogram (ERG) revealed evidence of unilateral macular dysfunction. Full-field ERGs revealed progressive global retinal dysfunction over 6 months, with unilateral decreases in amplitude and implicit time shifts, as seen in cases of autoimmune retinopathies. The eye eventually exhibited mild vitreous cellular infiltration on ophthalmoscopic examination, and vitrectomy diagnosed B cell non-Hodgkin’s lymphoma. Further evaluation revealed no evidence of central nervous system or systemic disease, consistent with occult PIOL.

Conclusions

This case illustrates an atypical presentation of PIOL characterized by unilateral retinal disease presenting with symptoms and signs of macular dysfunction. Clinical and ERG features evolved into an acute zonal occult outer retinopathy (AZOOR)-like phenotype. PIOL should be considered in atypical cases of AZOOR with vitreal reactions, and some cases of AZOOR may be related to B cell lymphocyte disorders.

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Acknowledgments

AG is funded by the Doris Duke Clinical Research Fellowship for medical students. Imaging is supported by the NEI Core grant 5P30EY019007, NCI Core grant 5P30CA013696, and unrestricted funds from Research to Prevent Blindness, New York, NY. DCH is supported by EY09076. The Bernard and Shirlee Brown Glaucoma Laboratory is supported by R01EY018213, the Foundation Fighting Blindness, Schneeweiss Stem Cell Fund, and the Tistou and Charlotte Kerstan Foundation. SHT is a member of the RD-CURE Consortium, Fellow of the Burroughs-Wellcome Program in Biomedical Sciences and has been supported by the Bernard Becker Association of University Professors in Ophthalmology Research to Prevent Blindness Award, Dennis W. Jahnigen Award of the American Geriatrics Society, Joel Hoffman Scholarship, Barbara and Donald Jonas Family Fund, and Professor Gertrude Rothschild Stem Cell Foundation.

Conflict of interest

None.

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Authors and Affiliations

  1. Department of Ophthalmology, Manhattan Eye, Ear and Throat Hospital, New York, NY, USA

    Gaetano R. Barile

  2. Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University, 635 West 165th Street, Room 218, New York, NY, 10032, USA

    Aakriti Garg, Donald C. Hood & Stephen H. Tsang

  3. Department of Psychology, Columbia University, New York, NY, USA

    Donald C. Hood

  4. Ophthalmic Oncology Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY, 10065, USA

    Brian Marr

  5. Department of Pathology, Columbia University, New York, NY, USA

    Shafinaz Hussein

  6. Bernard and Shirlee Brown Glaucoma Laboratory, Department of Pathology and Cell Biology, Columbia University, New York, NY, USA

    Stephen H. Tsang

Authors
  1. Gaetano R. Barile
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  2. Aakriti Garg
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  3. Donald C. Hood
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  4. Brian Marr
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  5. Shafinaz Hussein
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  6. Stephen H. Tsang
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Corresponding author

Correspondence to Aakriti Garg.

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Barile, G.R., Garg, A., Hood, D.C. et al. Unilateral retinopathy secondary to occult primary intraocular lymphoma. Doc Ophthalmol 127, 261–269 (2013). https://doi.org/10.1007/s10633-013-9409-7

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  • DOI: https://doi.org/10.1007/s10633-013-9409-7

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