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Manfred Zierhut, Rupesh Vijay Agrawal, Denise Carbonell, Sarakshi Mahajan, Tanja Berger, Bianka Sobolewska, Vishali Gupta, Soon Phaik Chee; Recommendations for the Diagnostic of vitreoretinal Lymphoma (VRL). Invest. Ophthalmol. Vis. Sci. 2019;60(9):3544.
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© ARVO (1962-2015); The Authors (2016-present)
To find recommendations for improving the diagnosis of vitreoretinal lymphoma, the ocular disease with the shortest survival rate.
Recommendations are based on a questionnaire (Delphi 1 round) with questions regarding clinical symptoms and signs, diagnostic procedures and imaging methods. The published literature was reviewed for evidence of clinical symptoms and signs and diagnostic laboratory and ophthalmic imaging investigations which support the diagnosis of VRL. This information was summarized and sent out with the questionnaire. Participants were mostly international uveitis specialists (n=28). This data was entered on-line and subsequently analyzed. In the second round (Delphi 2) items of the questionnaire not reaching consensus (75% agreement) in the first round, were discussed when 22 specialists, including a neuro-oncologist and 2 ocular-oncologists met in Tel Aviv.
Majority of patients over 50 years. Presenting symptoms are floaters and painless loss of vision without redness or photophobia.Predominantly bilateral but may present initially as unilateral. KP type may vary. Vitreous cells organized into sheets or clumps of any severity increase the suspicion of VRL. Eyes 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. Massive vitreous infiltration without macular edema is the most likely presentation. Diagnostic vitrectomy using low cut rate should be done after discontinuing systemic corticosteroid (where applicable) at least 2 weeks prior. Immediate analysis of fresh vitreous sample should be done. An IL 10:IL 6>1, positive MYD88 gene mutation and L265P mutation are strong indicators of VRL.Multi-modal imaging including spectral domain optical coherence tomography, fundus autofluorescence, fluorescein and indocyanine green angiography and B scan ultrasound are recommended when VRL is suspected. Contrast-enhanced MRI of brain is an essential investigation. PET CT scan of the whole body will exclude systemic involvement.
A consensus meeting allowed the establishment of statements and recommendations important for the diagnosis of VRL. This should contribute to earlier diagnosis and better chance of complete remission treatment.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.
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