15 years of PVRL‐experience of Prague uveitis Centre

Abstract

AbstractIntravitreal lymphoma is a malignant masquerade syndrome which is crucial to identify among uveitis patients because of its life‐threatening nature.A total of 35 immunocompetent patients, 15 men and 20 women, were observed with vitreoretinal lymphoma in the Centre for Diagnosis and Treatment of Uveitis in Prague between the years 2003 to 2019. Primary vitreoretinal lymphoma (PVRL) was diagnosed in 15 patients, 6 patients from this group did not show any CNS involvement nor had an intraocular relapse during the observation period. The clinical manifestation of lymphoma in our patients was usually the vitreous haze, sometimes accompanied by retinal lesions and anterior chamber cells with or without keratic precipitates. In our observation, the diagnosis was determined by cytology or/and flow cytometry from vitreous analysis in all patients with exception of 4 patients with clinically highly suspicious finding of vitreous. Beside the vitritis, retinal infiltration was present in 3 of these 4 patients. From this group of 4 patients, the diagnosis was confirmed from brain biopsy in 3 patients and lung biopsy in 1 patient. The vitreous analysis was negative for lymphoma in 2 patients and in 2 patients' vitreous analysis was not performed. Over the years, the sensitivity of VRL detection from vitreous sample became very high, in our observation 94%.All patients diagnosed with VRL by vitreous analysis were sent to haemato‐oncologist work‐up. Treatment was decided by the consensus of a haemato‐oncologist and an ophthalmologist according to the clinical manifestation. Combination of intravitreal and systemic chemotherapy is recommended in many reports. There has been a decline in ocular and brain radiotherapy in recent years because of the unpredictable and irreversible nature of its side effects. Autologous stem cell transplantation (ASCT) following systemic chemotherapy was indicated in 3 patients. The relapse of lymphoma was observed in 13 patients, first relapse was after 11–120 months (median 32 months).Supported by research project AZV MZ CR NU20‐03‐00253.