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Review
. 2017 Aug 18;10(8):1301-1307.
doi: 10.18240/ijo.2017.08.19. eCollection 2017.

Intraocular lymphoma

Affiliations
Review

Intraocular lymphoma

Li-Juan Tang et al. Int J Ophthalmol. .

Abstract

Intraocular lymphoma (IOL) is a rare lymphocytic malignancy which contains two main distinct forms. Primary intraocular lymphoma (PIOL) is mainly a sub-type of primary central nervous system lymphoma (PCNSL). Alternatively, IOL can originate from outside the central nervous system (CNS) by metastasizing to the eye. These tumors are known as secondary intraocular lymphoma (SIOL). The IOL can arise in the retina, uvea, vitreous, Bruch's membrane and optic nerve. There are predominantly of B-cell origin; however there are also rare T-cell variants. Diagnosis remains challenging for ophthalmologists and pathologists, due to its ability to masquerade as noninfectious or infectious uveitis, white dot syndromes, or occasionally as other metastatic cancers. Laboratory tests include flow cytometry, immunocytochemistry, interleukin detection (IL-10: IL-6, ratio >1), and polymerase chain reaction (PCR) amplification. Methotrexate-based systemic chemotherapy with external beam radiotherapy and intravitreal chemotherapy with methotrexate are useful for controlling the disease, but the prognosis remains poor. Therefore, it is important to make an early diagnose and treatment. This review is focused on the clinical manifestations, diagnosis, treatment and prognosis of the IOL.

Keywords: central nervous system; diagnosis; intraocular lymphoma; prognosis; treatment.

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Figures

Figure 1
Figure 1. Histopathological and immunohistological of IOL origining from B cell
The atypical cells have pleomorphic nuclei with conspicuous nucleoli and scanty cytoplasm (A, HE staining, 200×). It is positive for CD20 (B, 200×) and negative for CD3 (C, 200×); the high Ki-67 positive rate (average >80%) indicates extensive proliferation (D, 200×).
Figure 2
Figure 2. Histopathological and immunohistological of IOL origining from T cell
Medium-to-large-sized lymphoid cells with atypical nuclei are visualized with HE staining (A, 200×) and identified as CD3 positive (B, 200×), CD20 negative (C, 200×); the high Ki-67 positive rate (average >80%) indicates extensive proliferation (D, 200×).
Figure 3
Figure 3. Cytology of the vitreous specimen reveals some atypical, large lymphoid cells, with large, deeply stained, irregular nuclei and coarse chromatin (A, 400×), which is identified as CD20 positive (B, 400×).

References

    1. Buggage RR, Chan CC, Nussenblatt RB. Ocular manifestations of central nervous system lymphoma. Curr Opin Oncol. 2001;13(3):137–142. - PubMed
    1. Fredrick DR, Char DH, Ljung BM, Brinton DA. Solitary intraocular lymphoma as an initial presentation of widespread disease. Arch Ophthalmol. 1989;107(3):395–397. - PubMed
    1. Levy-Clarke GA, Buggage RR, Shen D, Vaughn LO, Chan CC, Davis JL. Human T-cell lymphotropic virus type-1 associated T-cell leukemia/lymphoma masquerading as necrotizing retinal vasculitis. Ophthalmology. 2002;109(9):1717–1722. - PubMed
    1. Goldey SH, Stern GA, Oblon DJ, Mendenhall NP, Smith LJ, Duque RE. Immunophenotypic characterization of an unusual T-cell lymphoma presenting as anterior uveitis. A clinicopathologic case report. Arch Ophthalmol. 1989;107(9):1349–1353. - PubMed
    1. Saga T, Ohno S, Matsuda H, Ogasawara M, Kikuchi K. Ocular involvement by a peripheral T-cell lymphoma. Arch Ophthalmol. 1984;102(3):399–402. - PubMed

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