T- cell Lymphoma and the Unusual Infiltrative Optic Neuropathy

Mycosis fungoides (MF) is a type of T-cell lymphoma representing 50% of all primary cutaneous lymphomas. The disease is usually limited to the skin, resembling eczema or psoriasis. Progressive vision loss and optic atrophy has been reported with tumor infiltrating the optic nerve, however, is uncommon. Here, we are reporting a rare case of MF offending the intracanalicular portion of the optic nerve causing acute no light perception.

Poster 200 T- cell Lymphoma and The Unusual Infiltrative Optic Neuropathy Zhenyang Zhao1, Andrew Whyte1, Dan Gombos1, Nagham Al-Zubidi1 1 The University of Texas MD Anderson Cancer Center, Houston, Texas, USA Introduction: Mycosis fungoides (MF) is a type of T-cell lymphoma representing 50% of all primary cutaneous lymphomas. The disease is usually limited to the skin, resembling eczema or psoriasis. Progressive vision loss and optic atrophy has been reported with tumor infiltrating the optic nerve, however, is uncommon. Here, we are reporting a rare case of MF offending the intracanalicular portion of the optic nerve causing acute no light perception. Description of Case(s): A 65-year-old male with a past medical history of epidermotrophic MF and MALT cell lymphoma presented with acute painless left eye (OS) vision loss for 2 days that progressed to no light perception. Ophthalmologic examination revealed a visual acuity of 20/20 OD and NLP OS with a left relative afferent pupillary defect. Fundus exam showed bilateral disc edema. MRI orbit revealed abnormal enhancement and T2 hyperintense signal within the retrobulbar and intracanalicular segments of the left optic nerve. Lumbar puncture with CSF analysis showed atypical T cells. Biopsy of the floor of the mouth showed CD4 positive peripheral T cell lymphoma with a high proliferation index. Flow cytometry was positive for T-cell. Given the clinical, radiographic and pathologic evidence for lymphoma, the patient was started urgently on intravenous steroid and radiation therapy to the bilateral orbits and brain. Follow up exam revealed resolution of previously noted disc edema along with the resolution of optic nerve enhancement on repeated MRI. The visual acuity OS improved to light perception. Conclusions, including unique features of the case(s): Although rare, T-cell lymphoma can infiltrate the optic nerve. Our case is unique as it is the first reported case in English literature that progressed to no light perception. Aggressive treatment with high dose steroids in conjunction with radiation therapy should be initiated in a timely manner to improve prognosis. References: None. Keywords: optic neuropathy, tumors Financial Disclosures: The authors had no disclosures. Grant Support: None. Contact Information: None provided. 200 | North American Neuro-Ophthalmology Society