Much more is known about effects on the eye from non-Hodgkin lymphoma than Hodgkin’s disease. Ocular complications are rare with Hodgkin lymphoma (HL), but may indicate a toxic reaction to systemic medications during therapy, or can point to a relapse in patients previously diagnosed with HL.
Investigators in Argentina and the US conducted a comprehensive review of studies describing ocular manifestations from HL, published from 1943 to 2019, according to a report in Ocular Oncology and Pathology. A total of 30 cases emerged, corresponding to 4 categories: inflammatory reaction, direct infiltration, paraneoplastic responses, and toxic drug effects.
Median age at presentation was 27 years. HL was diagnosed only after ocular signs in 10 patients, 33% of cases. For 3 with previous HL diagnosis, recurrence was discovered subsequent to eye-related symptoms.
Uveal inflammation was associated with HL most frequently, affecting 66% of those with ocular signs. Of the 21 with granulomatous uveitis, 3 patients experienced primarily anterior chamber inflammation, 13 displayed choroidal or retinal lesions, 7 showed optic disc swelling, and 5 had vitritis. Eight exhibited cotton-wool-like or hypopigmented chorioretinal spots. However, of 4 biopsied, no invasion by HL cells was detected. Uveitis partially resolved with corticosteroids for some, and improved with chemotherapy overall. Conjunctival or corneal inflammation was found in 3 individuals, 2 of which had nodular scleral lesions including necrotizing granulomas. Three other cases presented retinal vascular inflammation; frosted branch angiitis or periphlebitis, but responded well to chemotherapy, radiation, or ocular-indicated oral corticosteroids.
Researchers found 7 cases of optic nerve infiltration — all with HL stage 3B — and 1 case of vitreoretinal invasion. Four of those with optic nerve involvement showed a swollen disc with decreased visual acuity (VA) or visual field. The vitreoretinal patient was 73 years of age, with signs including granulomatous anterior uveitis, hazy vitreous, diminished VA, and posterior synechia. She was diagnosed with HL 1.5 years later and died in a few months — autopsy revealed Reed-Sternberg cells in the anterior chamber and trabecular meshwork.
“Importantly, the presence of Hodgkin/Reed-Sternberg-like cells alone is insufficient to establish a diagnosis of classic HL since these cells can occur in some types of non-HLs including peripheral T-cell lymphomas,” the study explains. Biopsy-shown tumor cell invasion was documented in just 1 case of the review.
In that case, a 24-year-old woman with stage 3B disease was diagnosed with paraneoplastic retinopathy after presenting with night blindness, mild vitritis, and diffuse paraneoplastic retinal pigment epithelium (RPE) atrophy. Chemotherapy succeeded in HL remission, but even with oral corticosteroids, VA did not improve. Another 31-year-old individual displayed macular edema and cotton-wool exudates distributed across the retina. This patient was diagnosed with a toxic reaction to a combination of drugs, and later succumbed to a secondary infection. Autopsy uncovered total atrophy of her sensory retina. One other case of immune checkpoint inhibitor, CPI-related uveitis was reported.
Studies in languages other than English were reviewed by abstract only. A limitation of the literature review is that ocular reactions from HL are rare — although a strength is the 76-year research period. “Overall prognosis is good” for patients with HL, the analysis noted.
Disclosures: Some study authors declared affiliations with the biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.
Valenzuela J, Echegaray JJ, Dodds E, et al. Ophthalmic manifestations of hodgkin lymphoma: a review. Ocul Oncol Pathol. 2021;7(6):381-389. doi:10.1159/000519032