Neuropathy and monoclonal gammopathy.
The association of neuropathy with monoclonal gammopathy has been known for several years, even if the clinical and pathogenetic relevance of this association is not completely defined. This is not a marginal problem since monoclonal gammopathy is present in 1-3% of the population above 50 years in whom it is often asymptomatic, and in at least 8% of patients is associated with a symptomatic neuropathy, representing one of the leading causes of neuropathy in aged people. Monoclonal gammopathy may result from malignant lymphoproliferative diseases including multiple myeloma or solitary plasmocytoma, Waldenström's macroglobulinemia (WM), other IgM-secreting lymphoma or chronic lymphocytic leukemia, and primary systemic amyloidosis (AL). In most instances it is not associated with any of these disorders and is defined monoclonal gammopathy of undetermined significance (MGUS) for its possible, though infrequent, evolution into malignant forms. Several data support the pathogenetic role of the monoclonal gammopathy in the neuropathy particularly when of IgM isotype where IgM reactivity to several neural antigens has been reported. Increased levels of VEGF have been implicated in POEMS syndrome. However, there are as yet no defined therapies for these neuropathies, as their efficacy has not been confirmed in randomized trials.
2nd Neurology, Department of Translational Medicine, Milan University, IRCCS Humanitas Clinical Institute, Rozzano, Milan, Italy. Electronic address: eduardo.nobile@unimi.it.
Eduardo
E
Nobile-Orazio
D000888:Antibodies, Anti-Idiotypic / Q000097:blood; D001798:Blood Proteins / Q000276:immunology / Q000378:metabolism; D006801:Humans; D010265:Paraproteinemias / Q000097:blood / Q000276:immunology; D010523:Peripheral Nervous System Diseases / Q000097:blood / Q000276:immunology
D000888:Antibodies, Anti-Idiotypic; D001798:Blood Proteins
IgM; MAG; Neuropathy; POEMS; antibodies; immunoglobulin; monoclonal gammopathy; plasma exchange; rituximab; sulfatide