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. 2009 Aug;133(8):1228-32.
doi: 10.5858/133.8.1228.

Primary intraocular lymphoma

Lisa J Faia  1 , Chi-Chao Chan
Affiliations

Primary intraocular lymphoma

Lisa J Faia et al. Arch Pathol Lab Med. 2009 Aug.

Abstract

Primary intraocular lymphoma, recently suggested to be renamed primary retinal lymphoma, is a subset of primary central nervous system lymphoma and is usually an aggressive diffuse large B-cell lymphoma. Between 56% and 85% of patients who initially present with primary intraocular lymphoma alone will develop cerebral lesions. Patients typically complain of decreased vision and floaters, most likely secondary to the chronic vitritis and subretinal lesions. The diagnosis of primary intraocular lymphoma can be difficult to make and requires tissue for diagnosis. The atypical lymphoid cells are large and display a high nuclear to cytoplasmic ratio, prominent nucleoli, and basophilic cytoplasm. Flow cytometry, immunohistochemistry, cytokine analysis, and gene rearrangements also aid in the diagnosis. Local and systemic treatments, such as chemotherapy and radiation, are employed, although the relapse rate remains high.

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Figures

Figure 1
Figure 1
Clinical presentations of primary retinal lymphoma. A, Slit lamp photography: sheets of vitreous cells seen in a patient later diagnosed with primary intraocular lymphoma (PIOL) via a diagnostic pars plana vitrectomy. B, Fundoscopy: active and inactive subretinal lesions throughout the fundus with areas of retinal necrosis in a patient diagnosed with PIOL. C, Fluorescein angiogram: areas of blockage (white arrows) of the retinal pigment epithelium and no signs consistent with inflammation in a patient with PIOL.
Figure 2
Figure 2
Cytology. A, Large and atypical lymphoid cells with large nuclei, prominent nucleoli, and basophilic cytoplasm found in the lumbar puncture specimen of a patient later diagnosed with primary intraocular lymphoma. B, Vitreous sample containing large lymphoid cells with large nuclei, prominent nucleoli, and basophilic cytoplasm (Giemsa, original magnifications ×1000 [A] and ×640 [B]).
Figure 3
Figure 3
Brain magnetic resonance imaging. Axial T2 imaging showing a 2-cm, heterogeneous lesion with surrounding inflammation in a patient diagnosed with primary intraocular lymphoma via a lumbar puncture specimen.
Figure 4
Figure 4
Gel electrophoresis of the polymerase chain reaction product of a patient with primary intraocular lymphoma (PIOL) showing IGH gene rearrangement in the complementarity-determining region 3, consistent with PIOL. Lane 1, patient with PIOL; lane 2, negative control; lane 3, positive control.
See this image and copyright information in PMC

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