Ocular Lymphoma in Patients with Systemic (Non CNS) Lymphoma: Unusual Presentations.
Diva R Salomao, Jose S Pulido, Patrick B Johnston, Irene C Fontcuberta, Andrew L Feldman. Mayo Clinic, Rochester, MN
Background: Intraocular lymphoma is rare. The primary form is considered a subset of primary CNS lymphoma and the secondary form is the result of ocular involvement in systemic lymphoma, which occurs in advanced stages of disease, often with concomitant CNS involvement. Vitreous-retinal infiltrates only are characteristic of the primary form, but are unusual without associated choroidal involvement in secondary ocular lymphoma. Furthermore, lymphoma transformation presenting in the eye is extremely rare, with only a few cases reported.
Design: Clinical history, cytological and histological preparations, immunostudies and outcome of 3 patients with systemic lymphoma and ocular involvement were reviewed.
Results: All patients were men, ages 54, 66 and 73 years, with complaints of blurred vision and floaters for several weeks before the diagnostic vitrectomy. Ophthalmic examination revealed clumps of vitreous cells, but no choroidal involvement. Patient 1 had no prior history of lymphoma; the diagnosis of ocular lymphoma prompted staging studies unveiling nodal disease a week later. Patient 2 carried the diagnosis of marginal zone lymphoma involving his bone marrow for 29 months, and patient 3, CLL for 7 months prior to the ocular symptoms. The diagnosis of secondary ocular lymphoma was based on the cytomorphological features and immunophenotype of the vitreous cells. Patients 1 and 3 had large B-cell lymphomas. Patient 2 showed clinical features consistent with transformation, but histological classification of the B-cell lymphoma was precluded by cellular degeneration. Patients 2 and 3 were found to have CNS involvement on MRI, 2 and 1 month following the diagnosis in the eye. All patients were treated with intraocular injections (rituximab, patient 1 and methotrexate, patients 2 and 3) in addition to systemic chemotherapy. All patients are alive; patient 1 without disease, and patients 2 and 3 with disease, after 22, 11 and 5 months follow-up after the diagnosis of ocular involvement.
Conclusions: We report 3 patients with systemic lymphoma and unusual ocular presentation. In one patient, the ocular symptoms and diagnosis preceded the systemic disease. In the other 2 patients with systemic low-grade lymphoma/leukemia, the ocular lymphoma was the presenting site of a higher grade process, consistent with transformation in the eye/CNS.
Wednesday, March 2, 2011 1:00 PM
Poster Session VI # 281, Wednesday Afternoon