Uveal lymphoma.

Abstract

Primary uveal lymphoma is rare and, unlike the vitreoretinal lymphomas, is typically indolent in nature [1– 3]. In the past these tumors were termed ‘reactive lymphoid hyperplasia’ or ‘uveal pseudotumor’ because of their lowgrade appearance [4– 6]. However, convincing evidence with modern techniques has demonstrated that the majority of these lesions are lowgrade Bcell lymphomas, most commonly extranodal marginal zone Bcell lymphomas [1, 5, 7] according to the current WHO classification (table 1) [8]. Primary highgrade lymphomas involving the uvea are extremely rare [26]. Uveal lymphoma may be considered primary if the uvea is the only or the initial site and secondary if there is secondary involvement of the uvea by systemic nonHodgkin lymphoma [13, 27– 34]. Hence, a careful clinical evaluation for involvement of other sites is required at the time of initial diagnosis and subsequent followup.