Investigation and Management of Immunoglobulin M- and Waldenström-Associated Peripheral Neuropathies

Oliver Tomkins; Veronique Leblond; Michael P Lunn; Karine Viala; Damien Roos Weil; Shirley D'Sa

doi:10.1016/j.hoc.2023.04.007

Investigation and Management of Immunoglobulin M- and Waldenström-Associated Peripheral Neuropathies

Hematol Oncol Clin North Am. 2023 Aug;37(4):761-776. doi: 10.1016/j.hoc.2023.04.007.

Authors

Oliver Tomkins¹, Veronique Leblond², Michael P Lunn³, Karine Viala⁴, Damien Roos Weil², Shirley D'Sa⁵

Affiliations

¹ Department of Haematology, Centre for Waldenströms Macroglobulinaemia and Related Conditions, University College London Hospitals NHS Foundation Trust, 250 Euston Road, London NW1 2PG, UK. Electronic address: https://twitter.com/tomkinsoliver.
² Department of Haematology, Sorbonne University and Pitié Salpêtrière Hospital, 47-83 Bd de l'Hôpital, Paris 75013, France.
³ National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.
⁴ Department of Clinical Neurophysiology, Sorbonne University and Pitié Salpêtrière Hospital, 47-83 Bd de l'Hôpital, Paris 75013, France.
⁵ Department of Haematology, Centre for Waldenströms Macroglobulinaemia and Related Conditions, University College London Hospitals NHS Foundation Trust, 250 Euston Road, London NW1 2PG, UK. Electronic address: s.dsa@nhs.net.

PMID: 37385714
DOI: 10.1016/j.hoc.2023.04.007

Abstract

The immunoglobulin M (IgM)-associated peripheral neuropathies (PN) are a heterogeneous group of disorders representing most paraproteinemic neuropathy cases. They are associated with IgM monoclonal gammopathy of undetermined significance (MGUS) or Waldenström macroglobulinemia. Establishing a causal link between a paraprotein and neuropathy can be challenging but is necessary to adopt an appropriate therapeutic approach. The most common type of IgM-PN is Antimyelin-Associated-Glycoprotein neuropathy, but half of the cases are of other causes. Progressive functional impairment is an indication for treatment, even when the underlying disorder is IgM MGUS, involving either rituximab monotherapy or combination chemotherapy to achieve clinical stabilization.

Keywords: Anti-MAG; CANOMAD; IgM neuropathy; Paraproteinemic neuropathy.

Publication types

Review

MeSH terms

Drug Therapy, Combination
Humans
Immunoglobulin M
Monoclonal Gammopathy of Undetermined Significance* / diagnosis
Monoclonal Gammopathy of Undetermined Significance* / therapy
Peripheral Nervous System Diseases* / diagnosis
Peripheral Nervous System Diseases* / etiology
Peripheral Nervous System Diseases* / therapy
Rituximab

Substances

Immunoglobulin M
Rituximab