50-year-old female had been experiencing blurry vision in both her eyes for three to four months
No other significant past ocular history
BCVA OD 20/25, OS 20/20, IOP normal in both eyes, no pupil abnormalities
Anterior segment slit lamp exam was normal
Chorioretinitis with vitritis
White dot syndromes
Previous lab work was carried out to reveal:
HLA-B27, HLA-B51, HLA-A29-Negative
Past medical history revealed that five years prior patient had underwent biopsy for a friable mass of her uterus due to abnormal vaginal bleeding
Prior Prior biopsy revealed: Diffuse infiltrates of large atypical lymphoid cells that expressed markers CD45, CD20, CD79a, PAX-5, MUM-1, BCL-6. These were consistent with diffuse large B-cell lymphoma
Patient underwent hysterectomy and systemic chemotherapy over a four month span five years ago
Patient had a PET-CT scan a few months prior to presentation demonstrating no suspicious uptake
Given that the laboratory work up was negative for any infectious or inflammatory markers, a diagnostic pars plana vitrectomy was carried out and showed the following:
Repeat full body PET-CT revealed hypermetabolic subcentimeter bilateral cervical and hypermetabolic and enlarged bilateral axillary lymph nodes concerning for malignancy
The vitreous biopsy showed abnormal lymphocytes staining positive for markers CD20, PAX-5, MUM-1, BCL-6, BCL-2, (CD3 negative) consistent with intraocular diffuse B-cell lymphoma with PET-CT demonstrating metastatic disease
Diffuse large B-cell lymphoma is the most common form of adult non-Hodgkins lymphoma and is the most frequent lymphoma subtype arising in the eye
The incidence of intraocular lymphoma represents less than 2% of ocular malignant tumors with overall incidence of 0.47 cases per 100,000 people per year
Co-expression of BCL6 and IRF4/MUM1 by CD20 positive cells in a vitreous biopsy is suggestive that B-cells are malignant
Staining of Ki-67 usually indicates high tumor cell growth
This patient’s disease is due to metastasis from previously treated lymphoma, likely finding safe harbor in the ciliary body for several years due to the unique vascularity of the tissue and relative immune privilege
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Kim et al., Survival outcomes of primary intraocular lymphoma: a single-institution experience. Am J Clin Oncol., 39: 109-113, 2016.
Korfel A and Schlegel U. Diagnosis and treatment of primary CNS lymphoma. Nat Rev Neurol 9: 317-327, 2013.
Sen HN, et al., Primary intraocular lymphoma: diagnosis and differential diagnosis. Ocul Immunol Inflamm, 17: 133-141, 2009.
Tang L-J, Gu C-L, Zhang P. Intraocular lymphoma. Int J Ophthalmol., 10: 1301-1307, 2017.
Damien Rodger, MD, PhD, Assistant Professor of Clinical Ophthalmology and Research Assistant Professor of Biomedical Engineering, firstname.lastname@example.org