Features
There are various types of intraocular lymphoma, including primary vitreoretinal lymphoma (PVRL), primary uveal lymphoma, and secondary uveal lymphoma. This chapter focuses on PVRL, which is the most common intraocular lymphoma. PVRL is a subtype of primary central nervous system large B-cell non-Hodgkin lymphoma (PCNSL). PVRL is usually diagnosed in the fifth to seventh decades of life, although it may present earlier, particularly in immunocompromised patients. About 300 cases are diagnosed per year in the United States. About two-thirds of patients with PVRL will present with bilateral involvement, and the vast majority will eventually exhibit bilateral disease. About 80% of patients with PVRL will develop PCNSL at some point during the course of the disease. In contrast, only 5 to 15% of patients with PCSNL will develop intraocular involvement.
95.1.1 Common Symptoms
May present with floaters, blurred vision, or be asymptomatic.
95.1.2 Exam Findings
The most characteristic clinical features include vitreous infiltration by white cells and creamy yellow sub-retinal pigment epithelium (sub-RPE) deposits (▶ Fig. 95.1). The vitreous cells often appear larger on slit lamp biomicroscopy than are typical of benign forms of vitritis. Less common clinical features include anterior chamber cells, keratic precipitates, pseudo-hypopyon, iris nodules, intra- or subretinal infiltrates, and choroidal infiltration. Rarely, PVRL can masquerade as an infectious retinitis.
Fig. 95.1 (a,b) Fundus photographs demonstrating typical vitritis (A > B) and sub-retinal pigment epithelium creamy yellow deposits of lymphoma cells that are characteristic of primary vitreoretinal lymphoma.
95.2 Key Diagnostic Tests and Findings
95.2.1 Optical Coherence Tomography
Optical coherence tomography (OCT) may aid in visualizing subretinal lesions and verifying anatomic location. Most OCT abnormalities are seen at the subretinal or RPE level. Characteristic small hyperreflective nodules at the RPE level are seen in some patients, which may dissipate with treatment.
95.2.2 Fluorescein Angiography or Ultra-Widefield Fluorescein Angiography
Fluorescein angiography (FA) or ultra-widefield fluorescein angiography findings are rarely diagnostic but can provide helpful confirmatory evidence to support the diagnosis of PVRL. The most common findings are diffuse granular RPE changes characterized by patchy hypofluorescence interspersed with pinpoint hyperfluorescence (▶ Fig. 95.2, ▶ Fig. 95.3). These are often out of proportion with the degree of RPE abnormality that is evidenced on ophthalmoscopic examination and color fundus photos. FA can also provide useful diagnostic information, often highlighting diffuse RPE changes that are less obvious clinically. Classic “leopard spot” hypofluorescent areas are perhaps the most characteristic finding.
Fig. 95.2 (a) Fluorescein angiography (FA) in an eye with primary vitreoretinal lymphoma (PVRL) showing diffuse granular retinal pigment epithelium changes characterized by patchy hypofluorescence interspersed with pinpoint hyperfluorescence. (b) FA in an eye with PVRL showing optic disc hyperfluorescence and leakage.
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