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Rare Case of Extramedullary Disease in Patient With Multiple Myeloma

By: Joseph Fanelli
Posted: Thursday, June 10, 2021

A case study presented in the Taiwan Journal of Ophthalmology (official publication of The Ophthalmological Society of Taiwan) highlighted the importance of recognizing extramedullary disease as a differential diagnosis of vision loss in patients with a history of multiple myeloma. The case also described an unusual location for the rare disease—the optic nerve head—which has rarely been described, noted Jason H. Peragallo, MD, of Emory University, Atlanta, and colleagues.

“The majority of extramedullary plasmacytomas arise from the walls of the upper respiratory tract, where they can spread directly to the orbits from the sinuses,” the authors noted. “Intraorbital disease tends to indicate a more aggressive course in comparison to extraorbital plasmacytomas.”

A 45-year-old woman reported gradual, progressive blurred vision following a diagnosis of optic neuritis 1 month earlier. The patient’s medical history included relapsed and refractory immunoglobulin A kappa multiple myeloma.

A dilated fundus examination revealed disc edema and hyperemia with splinter hemorrhage superiorly in the left eye; also noted was a small area of lobulated elevation on the nasal optic nerve head with blurring disc margin and obscuration of small vessels. This was thought to be a plasmacytoma of the optic nerve head.

A follow-up lumbar puncture revealed malignant plasma cells in the cerebrospinal fluid. The patient was then treated with palliative radiotherapy to the orbits, intrathecal methotrexate, and intravenous dexamethasone for 2 days. As a result, her visual acuity improved to 20/20 in the right eye and 20/400 in the left eye with a relative afferent pupillary defect. After electing follow-up treatment at a center closer to her home, the patient entered hospice and died 3 months later.

Disclosure: The authors reported no conflicts of interest.

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