Extranodal marginal zone lymphoma associated with Vogt-Koyanagi-Harada disease

Lymphoma associated with VKH disease

Authors

  • Timothy Kaftan University of Nebraska Medical Center Truhlsen Eye Institute
  • Helen Song Department of Ophthalmology, University of Miami, Miami, Florida, USA
  • Thomas Clare Department of Ophthalmology and Visual Sciences, University of Nebraska Medical Center, Omaha, NE, USA
  • Nam Nguyen Department of Ophthalmology and Visual Sciences, University of Nebraska Medical Center, Omaha, NE, USA
  • Rao Chundury Department of Ophthalmology and Visual Sciences, University of Nebraska Medical Center, Omaha, NE, USA
  • Jeffrey Cannatella Department of Pathology, Microbiology, and Immunology, University of Nebraska Medical Center, Omaha, NE, USA
  • Matthew Lunning Department of Internal Medicine, Division of Hematology/Oncology, University of Nebraska Medical Center, Omaha, NE, USA
  • Lindsay Helget Department of Internal Medicine, Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE, USA
  • Neha Gupta Department of Pathology, Microbiology, and Immunology, University of Nebraska Medical Center, Omaha, NE, USA
  • Steven Yeh Department of Ophthalmology and Visual Sciences, University of Nebraska Medical Center, Omaha, NE, USA

DOI:

https://doi.org/10.62856/djcro.v12.87

Keywords:

extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, lymphoma, melanocyte, uveitis, Vogt-Koyanagi-Harada disease

Abstract

This case report highlights a potential association between ocular lymphoma and recurrent uveal inflammation secondary to Vogt-Koyanagi-Harada (VKH) disease. A 61-year-old male with history of bilateral panuveitis was referred for management of exudative retinal detachment in the left eye (OS).  Visual acuity was 20/20 right eye (OD) and light perception OS. Funduscopic examination was significant for pigmentary changes OD and inferior exudative retinal detachment OS. The patient was diagnosed with VKH disease and was treated with topical steroids and mycophenolate mofetil. He later presented acutely with reduced vision to no light perception OS and intraocular pressure of 71 mmHg OS. A B-scan ultrasound OS showed presence of a posterior mass with an overlying retinal detachment and choroid thickening. Enucleation was performed. Histologic findings confirmed extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (EMZL). We hypothesize that EMZL may have evolved in the context of chronic and recurrent uveal inflammation.

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Published

2026-10-29

How to Cite

Kaftan, T., Song, H., Clare, T., Nguyen, N., Chundury, R., Cannatella, J., … Yeh, S. (2026). Extranodal marginal zone lymphoma associated with Vogt-Koyanagi-Harada disease: Lymphoma associated with VKH disease . Digital Journal of Case Reports in Ophthalmology, 12. https://doi.org/10.62856/djcro.v12.87

Issue

Vol. 12 (2026): Fall 2026

Section

Uveitis

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