What is the pathophysiology of ocular lymphoma?

Updated: Mar 15, 2019
  • Author: Manolette R Roque, MD, MBA, FPAO; Chief Editor: Hampton Roy, Sr, MD  more...
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Recent molecular studies demonstrating viral DNA in the ocular lymphoma cells suggest a role for infectious agents in the pathogenesis of intraocular lymphoma.

Extramarginal zone lymphomas, which account for most primary orbital and adnexal lymphomas, are characterized by an indolent natural history with frequent, continuous extranodal relapses. [7] Follicular lymphoma, diffuse large B cell lymphoma, mantle cell lymphoma, B-cell chronic lymphocytic leukemia, peripheral T-cell lymphoma, and natural killer cell lymphoma have also been reported to affect the orbit.

Several hypotheses explain the increasing incidence of non-Hodgkin lymphoma (NHL). Better imaging techniques, improved biopsy techniques, and newer classification systems are likely to have contributed to the apparent increase in the incidence of lymphoma. The aging population, the increasing number of immunosuppressive drugs, and the AIDS epidemic have also contributed to the increased incidence of NHL.

Chlamydia psittaci is associated with ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma, and this association varies across different geographical areas. [8, 9] In South Florida, orbital and adnexal lymphomas are not associated with C psittaci infections. [7]

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