Microvasculitic paraproteinaemic polyneuropathy and B-cell lymphoma.
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.
Turner
Martin R
MR
Department of Neurology, Institute of Psychiatry, King's College, De Crespigny Park, London SE5 8AF, UK. m.turner@iop.kcl.ac.uk
Warren
Jason D
JD
Jacobs
Jean M
JM
Groves
Mike J
MJ
Yong
Kwee
K
Honan
William P
WP
Thomas
P K
PK
Reilly
Mary M
MM
D000368:Aged; D017252:CD3 Complex / Q000378:metabolism; D022062:Electrophysiologic Techniques, Cardiac / Q000379:methods; D004727:Endothelium / Q000648:ultrastructure; D005205:Fascia; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000378:metabolism; D008297:Male; D008833:Microcirculation / Q000648:ultrastructure; D008854:Microscopy, Electron; D020422:Mononeuropathies / Q000209:etiology; D009431:Neural Conduction; D010265:Paraproteinemias / Q000150:complications* / Q000378:metabolism; D010266:Paraproteins / Q000378:metabolism; D011115:Polyneuropathies / Q000150:complications* / Q000378:metabolism; D012196:Review Literature as Topic; D014657:Vasculitis / Q000209:etiology*
D017252:CD3 Complex; C001437:IgM-L paraprotein; D007075:Immunoglobulin M; D010266:Paraproteins