Graefe’ s Arch Clin Exp Ophthalmol (2005) 243: 189–197 DOI 10.1007/s00417-004-0890-2 REVIEW ARTICLE Sarah E. Coupland Gerasimos Anastassiou Norbert Bornfeld Michael Hummel Harald Stein Received: 6 January 2004 Revised: 3 February 2004 Accepted: 9 February 2004 Published online: 10 December 2004 # Springer-Verlag 2004 Primary intraocular lymphoma of T-cell type: report of a case and review of the literature Abstract Purpose: Primary intraoc- ular lymphoma (PIOL) is an uncom- mon 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 immu- nocytological examination of vitrec- tomy 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 se- quencing were performed on the chorioretinal biopsy. Results: Cytol- ogy 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 se- quencing demonstrated a monoclonal amplification product within the ex- pected 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. Introduction Primary intraocular lymphoma (PIOL) is a rare non- Hodgkin lymphoma (NHL), first described in the literature in 1951 by Cooper and Riker as “reticulum cell sarcoma” [16]. PIOL can occur independently or in conjunction with primary central nervous system lymphoma (PCNSL), which is defined as a lymphoma limited to the craniospinal axis without evidence of systemic disease [24]. PIOL is considered a subset of PCNSL, and involves the retina, the vitreous and/or the optic nerve [55, 66]. It frequently pres- ents as a chronic, relapsing and steroid-resistant uveitis in elderly patients [2, 3, 6, 7, 26, 54, 66]. Young adults and children can, however, be affected [65, 73]. PIOL must be distinguished from (a) a secondary mani- festation of a systemic lymphoma in the eye, which tends to be distributed in the choroid [19, 43, 55]; and (b) a primary choroidal lymphoma with extension into the retina [4, 12, S. E. Coupland . M. Hummel . H. Stein Department of Pathology, Charité- Medical Faculty Berlin, Campus Benjamin Franklin, Berlin, Germany S. E. Coupland (*) Department of Pathology, Universitätsklinikum Benjamin Franklin, Hindenburgdamm 30, 12200 Berlin, Germany e-mail: secoupland@yahoo.de Tel.: +49-30-84453077 Fax: +49-30-84454473 G. Anastassiou . N. Bornfeld Department of Ophthalmology, University Hospital Essen, Essen, Germany