An apparent association between IgM paraproteinaemic neuropathy and glioblastoma, IDH-wildtype

What is it about?

A paraprotein (for example IgM) is an abnormal protein produced by plasma cells, which originate from B-cells. B-cells typically help fight infection. Peripheral neuropathy (nerve damage) is recognised as a complication of the presence of paraproteins in the blood. This is also associated with underlying haematological cancers such as lymphoplasmacytic lymphoma (an uncommon cancer of a type of B-cell which can produce a large amount of IgM) as well as other conditions such as IgM monoclonal gammopathy of clinical significance (spectrum of conditions with defined immunological and biochemical manifestations brought about by circulating IgM). Whilst it is well recognised that B cell disorders such as lymphoplasmacytic lymphoma can affect the nervous system by direct invasion of the cancer cells, there is no recognition of a link between such disorders and primary brain tumours such as glioblastoma, IDH-wildtype (primary). This case series presents three cases of classical IgM paraproteinaemic neuropathy who developed glioblastoma in the course of their illness following treatment with chemoimmunotherapy.

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Photo by Ash Edmonds on Unsplash

Photo by Ash Edmonds on Unsplash

Why is it important?

Both disorders are exceedingly rare, so it is highly unlikely that this is a random occurrence. It is unclear whether this probable association is due to common underlying risk factors for both diseases or due to immunosuppression from the treatment, and this merits further investigation.

Perspectives