Denise Carbonell1, Sarakshi Mahajan2, Soon-Phaik Chee1,3,4,5, Bianka Sobolewska6, Rupesh Agrawal7, Tanja Bülow8, Vishali Gupta9, Nicholas P Jones10, Massimo Accorinti11, Mamta Agarwal, Tracy Batchelor12, Jyotirmay Biswas13, Luca Cimino14, Ninette H tenDam-van Loon15, Alejandra de-la-Torre16, Shahar Frenkel17, Jacob Pe'er17, Michal Kramer18, Elisabetta Miserocchi19, Manabu Mochizuki20, Thomas Ness21, James T Rosenbaum22, H Nida Sen23, Michael Simion24, Helmut Sitter25, Daniel V Vasconcelos-Santos26, Zohar Habot-Wilner27, Sarah E Coupland28, José S Pulido29, Justine Smith30, Jennifer E Thorne31,32, Manfred Zierhut6. 1. Ocular Inflammation and Immunology section, Singapore National Eye Centre, Singapore. 2. St. Joseph Mercy Oakland, Pontiac, Michigan , United States. 3. Department of Ophthalmology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore. 4. Singapore Eye Research Institute, The Academia, Singapore. 5. Department of Ophthalmology & Visual Sciences Academic Clinical Program, Duke-NUS Medical School, Singapore. 6. Centre of Ophthalmology, University of Tuebingen, Tuebingen, Germany. 7. Department of Ophthalmology, National Healthcare Group Eye Institute, Singapore. 8. Department of Medical Statistics, RWTH Aachen University, Aachen, Germany. 9. Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India. 10. Uveitis service, Manchester Royal Eye Hospital, Manchester; Manchester Academic Health Science Centre, University of Manchester, Manchester, UK. 11. Department of Ophthalmology, Sapienza University of Rome, Rome, Italy. 12. Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts , United States. 13. Uveitis & Cornea Services, Sankara Nethralaya, Chennai, India. 14. Ocular Immunology Unit, Azienda USL-IRCCS, Di Reggio Emilia, Italy. 15. Department of Ophthalmology, University Medical Center Utrecht, Utrecht, The Netherlands. 16. NeURos Research Group, Escuela De Medicina Y Ciencias De La Salud, Universidad Del Rosario, Bogotá, Colombia. 17. Department Division of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. 18. Uveitis service, Department of Ophthamology, Rabin Medical Center, Sackler School of Medicine, Tel Aviv University, Tel Aviv-Yafo, Israel. 19. Ocular Immunology and Uveitis Service, University Hospital San Raffaele Milan, Milan, Italy. 20. Department of Ophthalmology & Visual Science Tokyo Medical and Dental University Graduate School, Miyata Eye Hospital, Miyakonojo, Miyazaki, Japan. 21. Department of Ophthalmology, University Eye Clinic Freiburg, Freiburg, Germany. 22. Uveitis Section, Oregon Health & Science and University Casey Eye Institute, Portland, Oregon, USA. 23. Uveitis Clinic, National Eye Institute, Bethesda, Maryland, USA. 24. Tel Aviv, Israel. 25. Institute of Surgical Research, Dept. Of Medicine, Philipps-University Marburg, Marburg, Germany. 26. Department of Ophthalmology, Uveitis section, Universidade Federal De Minas Gerais, Belo Horizonte, Brazil. 27. Division of Ophthalmology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv-Yafo, Israel. 28. Pathology Department, Liverpool Clinical Laboratories, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK. 29. Departments of Ophthalmology and Molecular Medicine, Mayo Clinic, Philadelphia, Pennsylvania, USA. 30. Eye and Vision Health section, Flinders University College of Medicine and Public Health, Adelaide, Australia. 31. Department of Ophthalmology, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. 32. Department of Epidemiology, Johns Hopkins University Bloomberg School of Public Health, Baltimore, Maryland, USA.
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.
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.
Authors: Justine R Smith; Alexandra L Farrall; Janet L Davis; Joke H de Boer; Anthony J Hall; Manabu Mochizuki; H Nida Sen; Hiroshi Takase; Ninette H Ten Dam-van Loon; Valérie Touitou; Daniel V Vasconcelos-Santos; David J Wilson; Steven Yeh; Mark H B Radford Journal: BMJ Open Date: 2022-07-28 Impact factor: 3.006
Authors: Benjamin Schrijver; P Martijn Kolijn; Josianne C E M Ten Berge; Nicole M A Nagtzaam; Angelique L C T van Rijswijk; Sigrid M A Swagemakers; Peter J van der Spek; Tom O A R Missotten; Mirjam E J van Velthoven; Joeri de Hoog; P Martin van Hagen; Anton W Langerak; Willem A Dik Journal: Acta Ophthalmol Date: 2021-07-28 Impact factor: 3.988