Blood-nerve barrier in IgM paraproteinemic neuropathy : a clinicopathologic assessment

We report the pathologic findings in a patient with sensorimotor neuropathy associated with Waldenström's macroglobulinemia, particularly in relation to blood-nerve barrier defects. The monoclonal IgM was of kappa type and possessed anti-HNK-1 activity. A sural nerve biopsy specimen revealed se...

Full description

Saved in:
Bibliographic Details
Published in:Acta neuropathologica 1998-02, Vol.95 (2), p.184-192
Main Authors: KANDA, T, USUI, S, BEPPU, H, MIYAMOTO, K, YAMAWAKI, M, ODA, M
Format: Article
Language:English
Subjects:
Aged
Autopsy
Biological and medical sciences
Biopsy
Central nervous system
Endothelial cells
Endothelium, Vascular - pathology
Endothelium, Vascular - ultrastructure
Female
Fibers
Hematologic and hematopoietic diseases
Humans
Immunoglobulin M
Immunoglobulin M - blood
Immunoglobulin M - cerebrospinal fluid
Intercellular Junctions - pathology
Intercellular Junctions - ultrastructure
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Lymphocytes B
Macroglobulinemia
Male
Medical sciences
Microscopy, Electron
Middle Aged
Nerve Fibers - pathology
Nerve Fibers - ultrastructure
Nervous system
Peripheral nerves
Peripheral neuropathy
Reference Values
Sensorimotor system
Spinal Cord - pathology
Spinal Nerve Roots - pathology
Sural nerve
Sural Nerve - blood supply
Sural Nerve - pathology
Sural Nerve - ultrastructure
Waldenstrom Macroglobulinemia - immunology
Waldenstrom Macroglobulinemia - pathology
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:We report the pathologic findings in a patient with sensorimotor neuropathy associated with Waldenström's macroglobulinemia, particularly in relation to blood-nerve barrier defects. The monoclonal IgM was of kappa type and possessed anti-HNK-1 activity. A sural nerve biopsy specimen revealed severe loss of myelinated and unmyelinated nerve fibers and gaps between adjacent endothelial cells of small endoneurial vessels. Postmortem findings 3 years later included severe loss of myelinated nerve fibers and diffuse infiltration by lymphoplasmacytic B cells throughout the peripheral nervous system, sparing the central nervous system. Findings in this case suggest an immune attack against endoneurial endothelial cells with permeation of IgM into peripheral nerve tissue.
ISSN:0001-6322
1432-0533
DOI:10.1007/s004010050785