Ocular non-Hodgkin's lymphoma: a challenge to diagnose and treat

Ocular non-Hodgkin's lymphoma has a variety of presentations and is difficult to diagnose. "The disease usually presents as a masquerade syndrome [that] is not well recognized at first," said Marc Dominique de Smet, MD, from the Department of Ophthalmology, Academic Medical Center, University of Amsterdam, The Netherlands.

"An important point is that clinicians realize how frequently ocular lymphoma has to be considered among patients in a uveitis referral clinic," Dr. de Smet said. "Overall, ocular lymphoma occurs in 2% of patients in the uveitis referral clinic."

Dr. de Smet noted that a high suspicion of the disease is needed to make the diagnosis and the realization that patients treated with uveitis are not responding to standard treatment. Ocular lymphoma usually presents in patients older than 60 years in most cases. Patients complain of central nervous system symptoms such as headaches, memory loss, and seizures. In patients with central nervous system lymphoma, ocular involvement occurs in 12% to 25% at present. The ocular presentation can be accompanied by good visual acuity considering the amount of inflammation present in the eye, with floaters and a slight decrease in visual acuity and a partial response to corticosteroids.

In the anterior segment the clinical features include mild anterior uveitis, iris infiltrates, and iris involvement with a thickened iris, nodules, and glaucoma development. In the posterior segment, vitritis is more characteristic; sub-retinal pigment epithelial infiltrates, vasculitis, and serous detachment occur. Biopsy yields a diagnosis in about 30% of cases.

During surgery, Dr. de Smet uses 20- and 23-gauge instrumentation and advised avoiding the smallest gauge instruments. Steroids should be stopped for 2 weeks before surgery. Undiluted specimens should be sent for analysis. Complete core vitrectomy should be performed with growth medium.

If a diagnosis cannot be established, he advised clinicians to wait for the reaccumulation of cells. The cells can be removed from the eye during a fluid gas exchange; this usually yields high-quality cells that facilitate a diagnosis. Other analytical methods include flow cytometry, comparison of interleukin measurements (IL) (IL-10 versus IL-6), and molecular techniques (gene rearrangements in IgH or bcl2/lgH translocation).