Abstract
P 043
Progredient uveal effusion in a patient with extranodal marginal zone B-cell lymphoma of the uvea: a case report
Heike Hasselbach1, Stefan Koinzer1, Ilske Oschlies2, Bernhard Nölle1, Jost Hillenkamp1, Johann Roider1
1Klinik für Ophthalmologie, Campus Kiel, Universitätsklinikum Schleswig-Holstein, Kiel; 2Sektion Hämatopathologie und Lymphknotenregister, Institut für Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Kiel
Objective
Primary intraocular lymphoma (PIOL) is a rare while often blinding disease with a poor prognosis. The diagnosis is difficult and often delayed because PIOL tends to mimics other ocular entities. The vast majority of PIOL arises in vitreous and retina and can be classified as high-malignant large B-cell lymphoma. Simultaneous or subsequent CNS-involvement occurs in up to 60-85% of patients. By contrast, only few cases with low-grade malignant extranodal marginal zone lymphomas confined to the choroidea have been described. The overall survival of this primary uveal lymphoma is very good with systemic disease and CNS involvement occurring only in a minority of patients.
Methods
Case report of a 78-year-old female patient with an extranodal marginal zone lymphoma of the uvea in the right eye initially diagnosed and treated as uveal effusion syndrome. Clinical, histological and immunhistochemical findings, echography and MRT images are presented.
Results
At the initial presentation the patient complained about a painless unilateral loss of vision progressive since four months. Decimal visual acuity (VA) was reduced to 0.4. Funduscopy revealed choroideal folds at the superior arcade and a circumscribed serous detachment of the central retina. A retrobulbar mass lesion could be ruled out by echography and MRT. After 50 months later VA was reduced to hand movements. Serous retinal detachment and prominent choroidal swelling occupying 9 clock hours had developed. The patient refused surgical intervention and biopsy despite rapid progression. 8 months later a blind and painful eye was enucleated. A dense infiltrate by small CD20 positive lymphocytes which were negative for CD10, Zyklin-D1 and CD5 with clonal cytoplasmatic light chain expression for kappa and negativity for lambda involved the entire uveal tract. The proliferation rate was < 5% (Ki-67).
Conclusions
Primary uveal extranodal marginal zone lymphoma and vitreoretinal high-malignant PIOL are lymphomas both occurring primarily in the eye but with different clinical pictures, courses and prognosis. Typical symptoms of primary uveal lymphoma are recurrent episodes of blurred vision, diffuse thickening of the uveal tract and metamorphopsia due to secondary serous detachment of the retina. It can easily be misdiagnosed as uveal effusion syndrome; diffuse, amelanotic melanoma, or posterior scleritis.
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