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Abdullah S. Alqahtani, Valerie Touitou, Nathalie Cassoux, Cedric Aknin, H. Merle-Beral, Phuc LeHoang, Bahram Bodaghi; More than a Masquerade Syndrome: Atypical Presentations of Primary Intraocular Lymphomas. Invest. Ophthalmol. Vis. Sci. 2011;52(14):4759.
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To present atypical manifestations of primary intraocular lymphoma (PIOL) that could mislead or delay diagnostic confirmation.
Patients with cytologically confirmed intraocular lymphoma referred between November 2009 and May 2010 in the Ophthalmology Department at the Pitié-Salpêtrière Hospital in Paris, France, were retrospectively reviewed. Clinical presentations of these patients were studied. Patients with severe vitritis and subretinal infiltrates or retinal pigment epithelium irregularities were considered to have typical manifestations of the disease and were excluded of the study. Diagnosis of PIOL was based on cytological examination of vitreous samples, with immunohistochemistry and molecular biology confirmation of the monoclonality using clonal IgH rearrangements examination. Viral, infectious and bacterial etiologies have been ruled out with PCR analysis of ocular fluids.
Twelve cases of cytologically proven PIOL were included during this period. Among those patients, 4 cases (2M/2F) were considered to be atypical in their presentations. Mean age was 54 year-old (range: 50-59 years). These patients presented with : an acute mydriasis and optic neuropathy in the absence of any infiltration of the posterior segment; a mild chronic auto-immune-like uveitis associated with epilepsy, a tuberculosis-like uveitis, and a pseudo-acute retinal necrosis syndrome. Progression of the disease was dramatically rapid with a poor visual outcome.
Diagnostic of primary intraocular lymphoma is very challenging and diagnostic delay in this condition is frequent. We describe patients with atypical forms of PIOL, characterized by a younger age, atypical features on examination and a possible fulminant evolution.
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