Purpose: To provide recommendations for diagnosis of vitreoretinal lymphoma (VRL). Methods: Literature was reviewed for reports supporting the diagnosis of VRL. A questionnaire (Delphi 1 round) was distributed to 28 participants. In the second round (Delphi 2), items of the questionnaire not reaching consensus (75% agreement) were discussed to finalize the recommendations. Results: Presenting symptoms include floaters and painless loss of vision, vitreous cells organized into sheets or clumps. Retinal lesions are usually multifocal creamy/white in the outer retina. Other findings include retinal lesions with “leopard-skin” appearance and retinal pigment epithelium atrophy. Severe vitreous infiltration without macular edema is the most likely presentation. Diagnostic vitrectomy should be performed. Systemic corticosteroid should be discontinued at least 2 weeks before surgery. An interleukin (IL)-10:IL-6 ratio > 1, positive mutation for the myeloid differentiation primary response 88 gene and monoclonality are indicators of VRL. Multi-modal imaging (optical coherence tomography, fundus autofluorescence) are recommended. Conclusions: A consensus meeting allowed the establishment of recommendations important for the diagnosis of VRL.

Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma / Carbonell, D.; Mahajan, S.; Chee, S. -P.; Sobolewska, B.; Agrawal, R.; Bulow, T.; Gupta, V.; Jones, N. P.; Accorinti, M.; Agarwal, M.; Batchelor, T.; Biswas, J.; Cimino, L.; tenDam-van Loon, N. H.; de-la-Torre, A.; Frenkel, S.; Pe'Er, J.; Kramer, M.; Miserocchi, E.; Mochizuki, M.; Ness, T.; Rosenbaum, J. T.; Sen, H. N.; Simion, M.; Sitter, H.; Vasconcelos-Santos, D. V.; Habot-Wilner, Z.; Coupland, S. E.; Pulido, J. S.; Smith, J.; Thorne, J. E.; Zierhut, M.. - In: OCULAR IMMUNOLOGY AND INFLAMMATION. - ISSN 0927-3948. - 29:3(2021), pp. 507-520. [10.1080/09273948.2021.1878233]

Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma

Cimino L.;
2021

Abstract

Purpose: To provide recommendations for diagnosis of vitreoretinal lymphoma (VRL). Methods: Literature was reviewed for reports supporting the diagnosis of VRL. A questionnaire (Delphi 1 round) was distributed to 28 participants. In the second round (Delphi 2), items of the questionnaire not reaching consensus (75% agreement) were discussed to finalize the recommendations. Results: Presenting symptoms include floaters and painless loss of vision, vitreous cells organized into sheets or clumps. Retinal lesions are usually multifocal creamy/white in the outer retina. Other findings include retinal lesions with “leopard-skin” appearance and retinal pigment epithelium atrophy. Severe vitreous infiltration without macular edema is the most likely presentation. Diagnostic vitrectomy should be performed. Systemic corticosteroid should be discontinued at least 2 weeks before surgery. An interleukin (IL)-10:IL-6 ratio > 1, positive mutation for the myeloid differentiation primary response 88 gene and monoclonality are indicators of VRL. Multi-modal imaging (optical coherence tomography, fundus autofluorescence) are recommended. Conclusions: A consensus meeting allowed the establishment of recommendations important for the diagnosis of VRL.
2021
29
3
507
520
Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma / Carbonell, D.; Mahajan, S.; Chee, S. -P.; Sobolewska, B.; Agrawal, R.; Bulow, T.; Gupta, V.; Jones, N. P.; Accorinti, M.; Agarwal, M.; Batchelor, T.; Biswas, J.; Cimino, L.; tenDam-van Loon, N. H.; de-la-Torre, A.; Frenkel, S.; Pe'Er, J.; Kramer, M.; Miserocchi, E.; Mochizuki, M.; Ness, T.; Rosenbaum, J. T.; Sen, H. N.; Simion, M.; Sitter, H.; Vasconcelos-Santos, D. V.; Habot-Wilner, Z.; Coupland, S. E.; Pulido, J. S.; Smith, J.; Thorne, J. E.; Zierhut, M.. - In: OCULAR IMMUNOLOGY AND INFLAMMATION. - ISSN 0927-3948. - 29:3(2021), pp. 507-520. [10.1080/09273948.2021.1878233]
Carbonell, D.; Mahajan, S.; Chee, S. -P.; Sobolewska, B.; Agrawal, R.; Bulow, T.; Gupta, V.; Jones, N. P.; Accorinti, M.; Agarwal, M.; Batchelor, T.; Biswas, J.; Cimino, L.; tenDam-van Loon, N. H.; de-la-Torre, A.; Frenkel, S.; Pe'Er, J.; Kramer, M.; Miserocchi, E.; Mochizuki, M.; Ness, T.; Rosenbaum, J. T.; Sen, H. N.; Simion, M.; Sitter, H.; Vasconcelos-Santos, D. V.; Habot-Wilner, Z.; Coupland, S. E.; Pulido, J. S.; Smith, J.; Thorne, J. E.; Zierhut, M.
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