The diagnosis and treatment of primary vitreoretinal lymphoma: A review

Jose S. Pulido, Patrick B. Johnston, Grzegorz S. Nowakowski, Allessia Castellino, Harish Raja

Research output: Contribution to journalReview articlepeer-review

26 Scopus citations


Background: To describe the recent diagnostic and treatment options for the most predominant form of primary vitreoretinal lymphoma (PVRL), namely diffuse large B cell lymphoma. This is mainly based on the experience at the Mayo Clinic as well as a partial review of the literature. MYD88 L265P mutation is seen in about 80% of cases; therefore, a polymerase chain reaction for this mutation helps in making the diagnosis that has been notoriously difficult to make. Local therapy using intravitreal methotrexate and rituximab has been very helpful in the treatment of the local disease. Systemic high-dose intravenous methotrexate is helpful in treating bilateral disease in conjunction with intravitreal therapy. Whether it is helpful in preventing or delaying the development of central nervous system lymphoma (CNSL) is still in dispute. If there is development of CNSL or recurrent ocular disease, alternatives to highdose methotrexate under investigation include pomalidomide, stem cell transplantation, or ibrutinib, with or without local therapy. Vitrectomy alone might be helpful as a debulking procedure. Because of the risks of redevelopment of disease, local radiation should be given if other options are not possible. Aqueous levels of IL10 are helpful in following the redevelopment of local disease. Conclusion: Although PVRL is still a difficult disease to diagnose and treat, new advances are helping to make these easier. Larger collaborative studies will be helpful in determining better treatments.

Original languageEnglish (US)
Article number18
JournalInternational Journal of Retina and Vitreous
Issue number1
StatePublished - 2018


  • Diffuse B cell lymphoma
  • Ibrutinib
  • MYD88 L265P
  • Vitreoretinal lymphoma

ASJC Scopus subject areas

  • Ophthalmology


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