Ocular adnexal lymphoma and intraocular lymphoma, whether occurring simultaneously or sequentially, are often similar to associated systemic lymphoma. We describe 4 cases of ocular adnexal lymphoma or intraocular lymphoma with a dissimilar systemic lymphoma. Two of the cases represent Richter transformation of chronic lymphocytic leukemia/small-cell lymphoma into diffuse large B-cell lymphoma. In the third patient, conjunctival extranodal marginal zone lymphoma developed following treatment for Hodgkin lymphoma. The fourth patient had a remote history of systemic diffuse large B-cell lymphoma with a subsequent diagnosis of orbital extranodal marginal zone lymphoma. Clinical-pathological correlation is reported for all cases in addition to pertinent review of the literature.
Keywords: chronic lymphocytic leukemia; diffuse large B-cell lymphoma; extranodal marginal zone lymphoma; intraocular lymphoma; non-Hodgkin's lymphoma; ocular adnexal lymphoma; small cell lymphoma.
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