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Primary intraocular lymphoma of T-cell type: report of a case and review of the literature

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Abstract

Purpose

Primary intraocular lymphoma (PIOL) is an uncommon non-Hodgkin lymphoma and is usually of B-cell type. Intraocular T-cell or T/NK-cell lymphomas are extremely rare and mostly represent a secondary manifestation of either a cutaneous or a systemic lymphoma. The aim of the current paper is to report the clinical, histopathological and molecular biological findings of a PIOL of T-cell type.

Methods

Conventional cytological and immunocytological examination of vitrectomy specimens. Conventional histology, immunohistochemistry and polymerase chain reaction (PCR) for the detection of immunoglobulin heavy chain (IgH) and T-cell-receptor gamma (TCR-γ) gene rearrangement, GeneScan analysis, and DNA sequencing were performed on the chorioretinal biopsy.

Results

Cytology of the right vitreous aspirate revealed a moderate cellular infiltrate consisting of medium-sized T-cells with pleomorphic nuclei. Similar atypical lymphocytes were seen in the partially necrotic chorioretinal biopsy. These lymphocytes expressed CD3, CD4, βF1 and CD30, with a growth fraction of 90%. TCR-γ-PCR, GeneScan analysis and DNA sequencing demonstrated a monoclonal amplification product within the expected range. In contrast, IgH-PCR revealed oligoclonal amplificates. The patient was treated with low-dose radiotherapy (total 45 Gy), and was in complete remission at final follow-up.

Conclusion

A rare PIOL of T-cell type was diagnosed on the basis of vitreous aspiration and chorioretinal biopsy. In addition to conventional cytology and immunocytology, the utilisation of gene rearrangement studies on vitreous or chorioretinal biopsies increases the chances of diagnosing or excluding a PIOL of either B-cell or T-cell type. Despite its rarity, ophthalmic pathologists should always consider the diagnosis of T-PIOL when reviewing vitreous samples.

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Authors and Affiliations

  1. Department of Pathology, Charité-Medical Faculty Berlin, Campus Benjamin Franklin, Berlin, Germany

    Sarah E. Coupland, Michael Hummel & Harald Stein

  2. Department of Pathology, Universitätsklinikum Benjamin Franklin, Hindenburgdamm 30, 12200, Berlin, Germany

    Sarah E. Coupland

  3. Department of Ophthalmology, University Hospital Essen, Essen, Germany

    Gerasimos Anastassiou & Norbert Bornfeld

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  1. Sarah E. Coupland
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  2. Gerasimos Anastassiou
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  4. Michael Hummel
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Correspondence to Sarah E. Coupland.

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Coupland, S.E., Anastassiou, G., Bornfeld, N. et al. Primary intraocular lymphoma of T-cell type: report of a case and review of the literature. Graefe's Arch Clin Exp Ophthalmol 243, 189–197 (2005). https://doi.org/10.1007/s00417-004-0890-2

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