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Erschienen in: Graefe's Archive for Clinical and Experimental Ophthalmology 11/2004

01.11.2004 | Review Article

Primary intraocular lymphoma: a review of the clinical, histopathological and molecular biological features

verfasst von: Sarah E. Coupland, Heinrich Heimann, Nikolaos E. Bechrakis

Erschienen in: Graefe's Archive for Clinical and Experimental Ophthalmology | Ausgabe 11/2004

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Abstract

Introduction

Primary intraocular lymphoma (PIOL) is a rare non-Hodgkin lymphoma which arises in the retina or the vitreous. It can occur either together with or independently of primary cerebral nervous system lymphoma (PCNSL); the incidence of the latter has significantly increased over the past three decades. PIOL remains one of the most difficult diagnoses to establish, particularly due to its ability to mimic other diseases in the eye and to the limited material which is often available for examination.

Methods

The article reviews the clinical, histopathological, molecular biological and biochemical approaches to the diagnosis of PIOL. The differential diagnoses, including other lymphomatous manifestations in the eye, e.g. primary uveal lymphoma, as well as non-neoplastic uveal diseases are addressed. Furthermore, the treatment strategies for PIOL are summarised.

Results

Diagnostic progress has been made in various fields, including flow cytometry and immunocytology, cytokine analysis, and as well as molecular biological analysis of the immunoglobulin heavy and light chains using polymerase chain reaction on both fixed and non-fixed material. The optimal therapy of PIOL remains to be determined: the current trends suggest that combined radiotherapy and chemotherapy, as well as intravitreal chemotherapy, are of value. Novel therapies which may have a role in the future include oral trofosfamide.

Conclusion

Our understanding of the pathogenesis of PIOL/PCNSL remains far from complete. Intensified efforts must be made to determine the cell of origin of PIOL, as well as to establish “molecular signatures”, which could be used to decrease diagnostic delay. Further studies, possibly prospective ones, are required to establish the optimal therapy for initial and recurrent disease.
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Metadaten
Titel
Primary intraocular lymphoma: a review of the clinical, histopathological and molecular biological features
verfasst von
Sarah E. Coupland
Heinrich Heimann
Nikolaos E. Bechrakis
Publikationsdatum
01.11.2004
Erschienen in
Graefe's Archive for Clinical and Experimental Ophthalmology / Ausgabe 11/2004
Print ISSN: 0721-832X
Elektronische ISSN: 1435-702X
DOI
https://doi.org/10.1007/s00417-004-0973-0

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