Immune-mediated Neuropathy with Anti-disialosyl IgM Antibodies in Diffuse Large B-cell Lymphoma: A Case Report and Literature Review

Abstract

A 36-year-old man with diffuse large B-cell lymphoma presented with polyneuropathy, and the diagnostic work-up revealed the presence of IgM antibodies against gangliosides with disialosyl residues (GD1b, GD3). He was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone and received high-dose intravenous immunoglobulin for the treatment of neuropathy. After initiating the treatments, the patient's neurological impairment improved dramatically. He currently remains in complete remission without a flare-up of the polyneuropathy. Based upon our experience and other case reports of lymphoma with immune-mediated neuropathy caused by anti-disialosyl ganglioside IgM antibodies, we conclude that determining the anti-ganglioside antibody profile and beginning early treatment should be considered promptly for patients with malignant lymphoma who develop polyneuropathy.

Diffuse large B-cell lymphoma involving peripheral nervous system with IgM antibodies against GM1 and GD1b

“…In general, the advanced tumors with higher stages intend to have a poorer prognosis and they are more likely to affect the nervous system. [8] Based on other reports of lymphoma with immune-mediated neuropathy caused by anti-disialosyl ganglioside IgM antibodies, we propose that the early diagnosis and treatment of both peripheral neuropathy and NHL may improve prognosis of patients. Therefore, it is generally considered that the treatments should focus on tumor treatment and immunomodulation, mainly through intravenous immunoglobulin or plasma exchange.…”

“…As the disease progresses, the result of electroneurography may have a change if it was tested again. Shihashi et al [8] summarized 6 cases of malignant lymphoma involving neuropathy with anti-disialosyl IgM antibodies in 2015 and 3 cases were diffuse large B-cell type. One case had IgM antibodies against GM1 and GQ1b in the serum.…”

Asymmetrical and Isolated Hypoglossal Nerve Palsy Accompanied by a New Subset of Anti-ganglioside Antibodies in a Patient with Diffuse Large B Cell Lymphoma

“…In patients with autoimmune neuropathy, anti-ganglioside IgM antibodies are associated with chronic disease, including malignant lymphoma, through the mechanism of ‘binding site drift’, while anti-ganglioside IgG antibodies are associated with acute diseases, including Guillain-Barré syndrome, through the mechanism of ‘molecular mimicry’ (7). Some previously reported cases of autoimmune neuropathy in malignant lymphoma were associated with anti-ganglioside IgM antibodies, including anti-GM1, anti-GD1b, and anti-GQ1b IgM antibodies (1), while cases with M protein were accompanied by anti-SGPG IgM antibodies (8, 9). Anti-GM1 IgM antibodies are related to an asymmetrical neuropathy, while anti-GQ1b and anti-SGPG IgM antibodies are usually associated with polyneuropathy (8, 9); one exception exists in a reported case of asymmetrical facial nerve palsy in a patient who was positive for anti-SGPG IgM antibodies (10).…”

“…The peripheral neuropathy observed in lymphoma is mainly due to the direct invasion of malignant cells, and sometimes due to a paraneoplastic syndrome associated with anti-ganglioside antibodies (1). Previous reports have shown five cases of asymmetrical hypoglossal nerve palsy in patients without malignancy who were positive for anti-ganglioside antibodies (Table) (2-6).…”

“…Malignant lymphoma mainly involves the peripheral nerves due to the direct invasion of malignant cells, and sometimes due to paraneoplastic syndrome associated with anti-ganglioside antibodies (1). However, to the best of our knowledge there have been no reported cases of lymphoma presenting with hypoglossal nerve palsy with anti-ganglioside antibodies.…”