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Clinical history of VRL
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The great majority of patients are 50 years or older. VRL tends to present with floaters and painless loss of vision. It tends not to cause redness or photophobia. VRL is predominantly bilateral but may present initially as unilateral.
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KPs of various types may be seen in VRL. Anterior synechiae and iris depigmentation are virtually never seen. Scleritis, pseudohypopon, hyphaema, anterior and posterior synechiae are uncommon.
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Vitreous cellular infiltration of any severity is usually seen in VRL. Cells organized into sheets or clumps increase the suspicion of VRL. Snowbanking, vitreous hemorrhage and retinal holes are unlikely signs. VRL patients with retinal lesions tend to show multifocal creamy/white lesions in the outer retina. Other findings include retinal lesions with leopard-skin appearance and RPE atrophy or fibrosis. Retinal hemorrhages, retinal vasculitis, macular edema, retinal detachment and necrotizing retinitis are unlikely, but may be features of advanced disease. Massive vitreous infiltration without macular edema is the most likely presentation for this condition.
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Systemic corticosteroids are not appropriate monotherapy for VRL. However typically, VRL improves to some extent on corticosteroids, but may worsen on dose reduction. If clinically feasible, systemic corticosteroids should be discontinued for at least 2 weeks before diagnostic vitrectomy to increase diagnostic yield.
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Diagnostic vitrectomy is the preferred investigation for those with clinical suspicion of VRL with vitreous involvement. AC tap for cytokine or gene analysis, where available, may assist diagnosis. Diagnostic vitrectomy with a low cut rate (up to 1500 cuts per minute) is recommended. An early undiluted vitreous sample may also be obtained. Undiluted and diluted vitreous should be immediately analyzed if possible. If not, there should be prior agreement with the pathologist on specimen preservation. Testing vitreous IL-10:IL- 6 is highly recommended. An IL10:IL6 ratio of greater than 1 is a strong indicator of VRL. Testing for MYD88 gene mutation on intraocular fluid is highly recommended. The L265P mutation is a strong indicator of VRL. It is recommended to use cell morphology, immunophenotype (flow cytometry/immunocytology/histochemistry), light chain restriction and IgH and/or TCR gene rearrangements (PCR). If vitreous cytology or flow cytometry are inconclusive after oncologic review by an expert cytopathologist, consider IL10:IL6 or MYD88 analysis, and if not available, consider chorioretinal biopsy or aspirate of suspicious lesions, or repeat vitrectomy. If cytology, flow cytometry, IL10:IL6 and MYD88 mutations are all negative, there is inadequate evidence to support further investigations for B cell lymphoma. If there is strong clinical suspicion of VRL but no supporting cytologic evidence, consider discussion with one of the following: a neurologist, hematologist or oncologist. In situations of diagnostic difficulty, a multidisciplinary approach may be helpful.
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Multi-modal imaging including spectral domain optical coherence tomography (OCT), fundus autofluorescence (FAF), fundus fluorescein angiography (FFA) and indocyanine green (ICG) angiography and B scan ultrasound are recommended when VRL is suspected. Contrast-enhanced MRI of brain is an essential investigation. CT/PET scan of the whole body will exclude systemic involvement. Hypofluorescent round lesions and well-defined hypofluorescent lesions corresponding to the infiltrates in the early and late phases (leopard-spots) on FFA are supportive of VRL. Granular hyper- and hypoautofluorescence pattern is supportive of VRL. Nodular hyperreflective lesions in or beneath the RPE are highly suggestive of VRL on SD-OCT. Widefield fundus photography is a good tool for documentation and follow-up.
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