Chemotherapy Effective for Rare Case of Lymphoma-linked NMOSD

O-CVP led to 'good' outcomes in woman with paraneoplastic NMOSD

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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A chemotherapy regimen was able to treat a woman’s neuromyelitis optica spectrum disorder (NMOSD) which was likely secondary to follicular lymphoma, a type of cancer that begins in cells of the immune system, according to a case report by researchers at King’s College London in the U.K.

O-CVP — a combination of the medications obinutuzumab, cyclophosphamide, vincristine, and prednisolone — is used to treat follicular lymphoma, but its use for NMOSD caused by such a cancer had never been reported.

“Treatment of our patient’s NMOSD, by treating the underlying follicular lymphoma with obinutuzumab, cyclophosphamide, vincristine and prednisolone, resulted in a good radiological and neurological outcome,” the researchers wrote.

Their report, “A novel approach using O-CVP to treat paraneoplastic NMO spectrum disorder associated with follicular lymphoma,” was published in the journal BMJ Case Reports.

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Cancer-linked NMOSD treated with three cycles of O-CVP

NMOSD is caused when an overactive immune system attacks some of the cells of the central nervous system, which is made up of the brain and spinal cord. This leads to damage of the spinal cord and the optic nerve, which carries messages from the eye’s retina to the brain.

Only rarely is NMOSD brought on by substances released by cancer cells that can make the immune system go out of control. When this happens, it is called paraneoplastic NMOSD.

Now, a team in the U.K. has reported the case of a woman in her 60s who received three cycles of treatment with O-CVP for her paraneoplastic NMOSD.

Induction treatment with O-CVP, followed by maintenance treatment, is recommended as an option for advanced follicular lymphoma, provided patients score a two or more on the Follicular Lymphoma International Prognostic Index (FLIPI). “The FLIPI score is used to guide treatment decisions,” the team wrote.

O-CVP may be used as therapeutic option in patients with NMOSD driven by follicular lymphoma

On a visit to the hospital, the woman said she had been experiencing weakness in her legs and a sensation of tingling and numbness for the past six weeks. Her belly felt bloated and she had lost some weight over the past month.

A physical examination revealed weakened hip flexors (the muscles located near the top of the thighs) and absent reflexes in her legs and feet.

An MRI scan of the spine revealed longitudinal extensive transverse myelitis, which occurs when the spinal cord shows damage over three or more vertebra. A brain MRI scan was clear.

A blood test came back positive for aquaporin-4 antibodies, which are self-reactive antibodies that cause most cases of NMOSD. Based on these findings, the woman was diagnosed with NMOSD.

As part of the workup for NMOSD, the woman also underwent positron emission tomography and CT scanning. The scan revealed that lymph nodes throughout her body had cells more active than normal, which grew as groups to form nodules.

A closer look into a piece of tissue from the lymph nodes revealed that the overly active cells were B-cells, a type of immune cell. Based on these findings, the woman also received a diagnosis of low-grade follicular lymphoma with a FLIPI score of 4.

Initial treatment was with high-dose intravenous (into-the-vein) methylprednisolone, followed by oral prednisolone. Both medications are corticosteroids.

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Patient undergoes plasma exchange after responding poorly to corticosteroids

Because the woman responded poorly to the corticosteroids, she underwent a five-day course of plasma exchange. During this procedure, a machine separates plasma (the clear, liquid part of the blood) from the blood and swaps it for a plasma substitute before the blood is returned to the body.

While the patient’s longitudinal extensive transverse myelitis eased, no significant clinical recovery was noted.

At this point, the team concluded the woman might have paraneoplastic NMOSD, and started her on O-CVP.

“A high index of suspicion is needed to identify patients with paraneoplastic NMOSD, which is more likely in elderly patients,” the team wrote.

After two cycles of O-CVP, the woman’s hip flexing and lower limb reflexes had improved, and the longitudinal extensive transverse myelitis had regressed further until it was barely visible on an MRI scan.

However, after the third cycle of O-CVP, the woman developed neutropenic sepsis, which occurs when the number of neutrophils (a type of white blood cell) drops too low and the immune system mounts a whole-body response to an infection. This is a known side effect of O-CVP, which led the woman to stop the treatment.

As she recovered from the neutropenic sepsis, she underwent 10 weeks of neurorehabilitation before being discharged home. At the time of writing of the report, she was on “community rehabilitation,” the team wrote.

Despite potential side effects, “O-CVP may be used as therapeutic option in patients with NMOSD driven by follicular lymphoma,” the researchers concluded.