Uveal Lymphoma

  • Mary E. Aronow
  • Chi-Chao Chan
Living reference work entry


Uveal lymphoma by definition affects the choroid, iris, and ciliary body. The vast majority of cases primarily involve the choroid. Uveal lymphoma can be further characterized as a primary uveal lymphoma or as a secondary intraocular manifestation of systemic lymphoma. Most cases of primary choroidal lymphoma (more than 80 %) are low-grade, extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT). The remainders are comprised of subtypes such as follicular, lymphoplasmacytic, small lymphocytic, mantel cell, diffuse large B-cell lymphoma, and others. Treatment depends upon the extent of ocular involvement, laterality, and presence or absence of systemic involvement. Therapies may include radiation, immunotherapy, and chemotherapy. Overall survival, particularly with low-grade, indolent forms such as EMZL, is excellent.


Anterior Chamber Exudative Retinal Detachment Choroidal Metastasis Lymphoplasmacytic Lymphoma Posterior Chamber Intraocular Lens 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Suggested Reading

  1. Aronow ME, Portell CA, Sweetenham JW, Singh AD. Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes. Ophthalmology. 2014;121:334–41.CrossRefPubMedGoogle Scholar
  2. Cheung MK, Martin DF, Chan CC, Callanan DG, Cowen CL, Nussenblatt RB. Diagnosis of reactive lymphoid hyperplasia by chorioretinal biopsy. Am J Ophthalmol. 1994;118:457–62.CrossRefPubMedGoogle Scholar
  3. Coupland SE, Damato B. Lymphomas involving the eye and the ocular adnexa. Curr Opin Ophthalmol. 2006;17:523–31.PubMedGoogle Scholar
  4. Coupland SE, Foss HD, Hidayat AA, Cockerham GC, Hummel M, Stein H. Extranodal marginal zone B cell lymphomas of the uvea: an analysis of 13 cases. J Pathol. 2002;197:333–40.CrossRefPubMedGoogle Scholar
  5. Fuller ML, Sweetenham J, Schoenfield L, Singh AD. Uveal lymphoma: a variant of ocular adnexal lymphoma. Leuk Lymphoma. 2008;49:2393–7.CrossRefPubMedGoogle Scholar
  6. Loriaut P, Charlotte F, Bodaghi B, Decaudin D, Leblond V, Fardeau C, et al. Choroidal and adnexal extranodal marginal zone B-cell lymphoma: presentation, imaging findings, and therapeutic management in a series of nine cases. Eye (Lond). 2013;27:828–35.CrossRefGoogle Scholar
  7. Mashayekhi A, Shukla SY, Shields JA, Shields CL. Choroidal lymphoma: clinical features and association with systemic lymphoma. Ophthalmology. 2014;121:342–51.CrossRefPubMedGoogle Scholar

Copyright information

© Springer (India) Pvt. Ltd. (outside the USA) 2016

Authors and Affiliations

  1. 1.Ophthalmic Oncology/Retina DivisionWilmer Eye Institute, Johns HopkinsBaltimoreUSA
  2. 2.Laboratory of ImmunologyNational Eye Institute, National Institutes of HealthBethesdaUSA

Personalised recommendations