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Microvasculitis may play a greater part in the pathogenesis of paraproteinaemic neuropathies than is generally recognised, producing tissue destruction by convergent immune and physical mechanisms. We present a patient with a clinical syndrome of mononeuritis multiplex and a circulating IgM lambda paraprotein, in whom bone marrow aspiration revealed a lymphoplasmacytoid lymphoma. Microvasculitic changes were present in the first nerve biopsy, and the second showed extensive destruction of neural architecture and deposition of IgM-related material. A 2-stage pathogenic cascade is postulated and explored with a review of the relevant literature.

Type

Journal article

Journal

J Peripher Nerv Syst

Publication Date

06/2003

Volume

8

Pages

100 - 107

Keywords

Aged, CD3 Complex, Electrophysiologic Techniques, Cardiac, Endothelium, Fascia, Humans, Immunoglobulin M, Lymphoma, B-Cell, Male, Microcirculation, Microscopy, Electron, Mononeuropathies, Neural Conduction, Paraproteinemias, Paraproteins, Polyneuropathies, Review Literature as Topic, Vasculitis