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Diagnosis of a primary uveal extranodal marginal zone B-cell lymphoma by chorioretinal biopsy: case report

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Abstract

Purpose

To report the clinical, histopathological and molecular biological findings of a primary extranodal marginal zone B-cell lymphoma (EMZL) of the uvea.

Methods

Conventional histology, immunohistochemistry and polymerase chain reaction for immunoglobulin heavy chain gene rearrangement (IgH-PCR) and GeneScan analysis were performed on a chorioretinal biopsy.

Results

Histological examination of the chorioretinal biopsy demonstrated a dense infiltrate of small centrocyte-like cells, plasmacytoid tumour cells and occasional blasts. The tumour cells were positive for CD20, showed monotypical expression for Ig-kappa and IgM, and a growth fraction of 10%. Clonality analysis using IgH-PCR disclosed a monoclonal B-cell population. Following localised irradiation of 35 Gy, a complete remission was achieved.

Conclusion

This report describes a rare uveal EMZL diagnosed following investigation of a chorioretinal biopsy. Despite its rarity, ophthalmic pathologists should consider the diagnosis of a primary uveal EMZL when reviewing chorioretinal biopsies.

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Correspondence to Sarah E. Coupland.

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Coupland, S.E., Joussen, A., Anastassiou, G. et al. Diagnosis of a primary uveal extranodal marginal zone B-cell lymphoma by chorioretinal biopsy: case report. Graefe's Arch Clin Exp Ophthalmol 243, 482–486 (2005). https://doi.org/10.1007/s00417-004-1050-4

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  • DOI: https://doi.org/10.1007/s00417-004-1050-4

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