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SYSTEMIC LYMPHOMA MASQUERADING AS BIRDSHOT CHORIORETINOPATHY.
Elizabeth Y Wei1, Marko M Popovic2, Panos G Christakis1,2,3
1Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
Retinal Cases & Brief Reports
|February 28, 2025
Summary
A rare case of birdshot-like chorioretinopathy (BSCR) occurred in an HLA-A29 negative patient with a history of lymphoma. Ocular findings remained stable despite the development of diffuse large B-cell lymphoma (DLBCL).
Area of Science:
- Ophthalmology
- Oncology
- Medical Genetics
Background:
- Birdshot-like chorioretinopathy (BSCR) is typically associated with the HLA-A29 allele.
- Systemic lymphoma can present with diverse ocular manifestations.
- Atypical presentations of BSCR warrant thorough investigation for underlying neoplastic or autoimmune conditions.
Purpose of the Study:
- To describe a rare instance of BSCR in a patient negative for HLA-A29.
- To investigate the potential link between systemic lymphoma and BSCR.
- To highlight the importance of considering masquerade syndromes in BSCR diagnosis.
Main Methods:
- Case report of a 58-year-old male with blurry vision and floaters.
- Clinical examination revealed bilateral atrophic birdshot-like choroidal lesions.
- Diagnostic work-up included HLA typing, infectious disease screening, MRI, and electroretinography (ffERG, mfERG).
Main Results:
- The patient was HLA-A29 negative, with no evidence of syphilis or tuberculosis.
- MRI showed cranial nerve enhancement, but no overt malignancy.
- Ocular findings remained stable over two years, despite a later diagnosis of diffuse large B-cell lymphoma (DLBCL).
Conclusions:
- BSCR masquerade syndromes should be considered in HLA-A29 negative patients with birdshot-like lesions, especially those with a history of lymphoma.
- The patient's chronic choroidal findings may be linked to prior low-grade B-cell lymphoma and chemotherapy.
- The relationship between choroidal involvement and transformation to high-grade lymphoma remains unclear.

