Unilateral retinopathy secondary to occult primary intraocular lymphoma

Gaetano R. Barile, Aakriti Garg, Donald C. Hood, Brian Marr, Shafinaz Hussein, Stephen H. Tsang

Research output: Contribution to journalArticlepeer-review

2 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)261-269
Number of pages9
JournalDocumenta Ophthalmologica
Issue number3
StatePublished - Dec 2013
Externally publishedYes


  • Full field electroretinogram
  • Multifocal electroretinogram
  • Non-Hodgkin's Lymphoma
  • Primary intraocular lymphoma
  • Progressive retinal dysfunction
  • Spectral domain optical coherence tomography

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Physiology (medical)


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