Abstract

SO.07.05

Primary intraocular lymphoma with dramatic therapeutic response and surprising intraocular findings following vitrectomy

Claudia Auw-Haedrich1, Annette Schmitt-Graeff3, Silke Lassmann3, Paul Fisch3, Martin Werner3, Thomas Ness1, Gerald Illerhaus2
1Universitäts-Augenklinik Freiburg, 2Institut für Pathologie, Universitätsklinikum Freiburg, 3Medizinische Universitäts-Klinik, Universitätsklinikum Freiburg

Introduction
Primary intraocular lymphoma (PIOL) is a rare but very important entity for ophthalmologists since they often play the initial role in diagnosing this life threatening disease. We present a case with unusual course.
Material and methods
The 72-year-old patient was diagnosed an acute retinal necrosis syndrome in the right eye. This eye underwent vitrectomy and oil instillation but finally went blind under systemic aciclovir treatment. Six months later vitreous infiltrates of the left eye occurred and the diagnosis primary intraocular lymphoma was confirmed histologically. The visual acuity of the only left eye dropped to hand motion despite chemotherapy and thus radiotherapy was performed. The visual acuity improved to 0.4 and is still 0.5 following cataract surgery in Mai 2003. In March 2003 multiple pulmonal and abdominal lymphomas were detected by computertomography as the only systemic manifestation of the PIOL, followed by another chemotherapy. In June 2008, when the patient was in full remission, the painful blind right eye was enucleated. Clinically the intraocular segment was obscured by corneal opacification.
Results
Histologically the retina of the enucleated eye was surprisingly completely exchanged by polymorphous lymphoma cells, which stained positively with antibodies against CD20, BCL-2, BCL-6 but negatively with antibodies against CD138. Inflammatory cells within the sclera stained different with some of them being CD138-positive as sign of plasmacellular differentiation. The patient underwent a therapy with intravenous rituximab.
Conclusion
The lymphoma cells detected within the blind eye seem to represent surviving lymphoma cells despite chemotherapies. The ACAID phenomenon could also have supported tumor cells survival. We assume that radiotherapy of the blind eye, when the left eye was irradiated would have been useful. This treatment could have eliminated these cells and possibly prevented the systemic spread causing the pulmonal and abdominal lymphomas.

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