MALT Lymphoma Presenting as Symptomatic Optic Neuritis: A Case Report (P14-13.001)

Abstract

<h3>Objective:</h3> To report a case of MALT lymphoma presenting as optic neuritis and highlight its inherent diagnostic challenges. <h3>Background:</h3> Optic neuritis (ON) is the inflammation of the optic nerve associated with multiple sclerosis,. ON with atypical clinical features require thorough evaluation. Primary ocular adnexal lymphomas are the most common orbital tumors, and IgG4-related ocular disease also presents with optic neuropathy and consists of morphologically similar immune infiltrates. Their diagnoses are challenging as corticosteroids can incur non-diagnostic biopsies, complicating further workup. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> 51-year-old healthy African American woman presented with one month of progressive left monocular blurry vision, color desaturation and_pain. Exam showed left visual acuity of 20/60, afferent pupillary defect, and 0/13 Ishihara plates identified in left eye. Remainder of exam was intact. MRI demonstrated left optic nerve sheath enhancement and enlargement. Another enhancing lesion was identified in the right inferior orbit. Despite visual impairment, corticosteroids were deferred prioritizing diagnostic tests. Workup was negative for autoimmunity, systemic neoplasm, and infection. CSF showed a bland profile without malignant cells. Serum and CSF IL2 receptor levels returned normal. Oligoclonal bands and IgG were elevated in serum and CSF; IgG4 levels were high at 156, raising concern for IgG4-related ocular disease. Right intra-orbital mass biopsy demonstrated atypical CD20+ lymphoid infiltrates with rare germinal centers, but &lt;10% plasma cells expressed IgG4. Further molecular analysis was positive for monoclonal IgH gene rearrangement, diagnosing mucosa-associated lymphoid tissue (MALT) lymphoma in the right orbit and probable left optic nerve involvement. She was initiated on bendamustine and rituximab with stabilization of her ocular symptoms. <h3>Conclusions:</h3> Atypical presentations of ON warrant thorough workup. IgG4-related ocular disease and ocular adnexal MALT lymphoma share similarities that require histologic and molecular analysis to differentiate. Early identification of MALT lymphoma is crucial to initiate proper therapies, maintain disease control, and preserve visual acuity. <b>Disclosure:</b> Dr. Jia has nothing to disclose. Ms. Wyckoff has nothing to disclose. Dr. Cohen has nothing to disclose. Dr. Bryar has nothing to disclose. Dr. Lukas has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck and Company, Inc.. Dr. Lukas has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Merck and Company, Inc.. Dr. Lukas has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Novocure. Dr. Lukas has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for EBSCO. Dr. Lukas has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Medlink Neurology. Dr. Lukas has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. The institution of Dr. Lukas has received research support from BMS. The institution of Dr. Lukas has received research support from BrainUp. The institution of Dr. Lukas has received research support from National Cancer Institute. The institution of Dr. Lukas has received research support from National Cancer Institute. The institution of Dr. Lukas has received research support from Ziopharm. Dr. Dixit has nothing to disclose.