Mantle cell lymphoma is a rare aggressive subtype of non-Hodgkins B cell lymphoma. It typically presents with asymptomatic monoclonal lymphocytosis, lymphadenopathy or bulky extranodal disease. Mantle cell lymphoma rarely affects the central nervous system. We present two cases in which vision loss was the initial symptom of central nervous system involvement by the malignancy.
Both patients initially received high dose intravenous steroids with notable improvement in their vision.
Conclusions and importance
Early detection and management of optic nerve infiltration by mantle cell lymphoma is essential as it improves visual outcomes and enables prompt management of the patient’s systemic disease.
Mantle cell lymphoma is a rare, aggressive subtype of non-Hodgkins B cell lymphoma representing 5–10% of all non-Hodgkins lymphoma. Patients can present with asymptomatic lymphocytosis, bulky extranodal disease (splenomegaly, lymphomatous polyposis in the gastrointestinal tract, kidney masses), or generalized lymphoadenopathy. Central nervous system (CNS) involvement in this condition is uncommon and present in 4–26% of cases. However once the CNS is involved, the disease has an extremely poor prognosis with a median survival of approximately 5 months. , Up to one quarter of individuals with nervous system involvement will have concomitant ophthalmic disease. Mantle cell lymphoma involving the ocular adnexa occurs in 1–4% of people and is more common in patients with disseminated disease. , Intraocular involvement has only been reported in 10 prior cases and has a propensity for uveal tissue presumably due to its rich vascular supply. , , Even less common is infiltration of the optic nerve, with only 3 prior reported cases in the literature. , , We present two cases of optic nerve infiltration with mantle cell lymphoma as the first sign of central nervous system involvement of the malignancy.
An 84 year-old man presented with a six month history of bilateral painless progressive visual decline. He endorsed poor energy, fatigue and loss of appetite over one month. He denied jaw claudication, scalp tenderness, myalgias, headaches and transient vision loss. He had a complex past medical history with a diagnosis of mantle cell lymphoma eight years prior treated with rituximab and bendamustine resulting in complete remission. The patient remained on maintenance rituximab every three months. He had a recurrence of his cancer two years prior to presentation with remission on subsequent cycles of rituximab.
On examination, the patient was frail and cachectic. His vision was hand motion in the right eye and count fingers at 1 foot in the left eye. He had a right relative afferent pupillary defect. Extraocular motility was normal. Slit lamp examination showed an unremarkable anterior segment. Fundoscopy revealed bilateral optic nerve swelling with a peripapillary hemorrhage inferior to the left optic nerve. The patient also had large soft drusen bilaterally in keeping with macular degeneration ( Fig. 1 ). General neurologic examination was normal. His physical exam was notable for a large, matted, nontender right preauricular lymph node.
Magnetic Resonance Imaging (MRI) with and without contrast demonstrated bilateral thickening of the pre-chiasmic optic nerves with associated diffuse enhancement and diffusion restriction of the optic nerves in keeping with lymphomatous infiltration ( Fig. 2 ). 18F- Fluorodeoxyglucose Positron Emission Tomography scan revealed numerous areas of hypermetabolic foci in the head, neck, thorax, renal parenchyma, and lymph nodes. Non-contrast Computed Tomography imaging of the chest abdomen and pelvis corroborated this with numerous areas of lymphadenopathy as well as nodular renal and biliary involvement. Cerebrospinal fluid analysis revealed 255 white blood cells (77% cells being atypical lymphocytes), elevated protein at 134, and normal glucose. Flow cytometry confirmed a monoclonal lymphomatous population within the cerebrospinal fluid (CD5 kappa B cell population) in keeping with mantle cell lymphoma. Treatment with intravenous steroids, intrathecal methotrexate, ibrutinib and rituximab was initiated with improvement in the patient’s vision. Unfortunately, four months following this presentation, the patient ultimately succumbed to his systemic illness.