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Published on: November 9, 2017
[Neuropathy in Waldenstrom's macroglobulinemia]
Acta Medica Portuguesa
|April 1, 1995
Summary
Waldenström's macroglobulinaemia can present as a first sign of a progressive sensory neuropathy. Nerve biopsy showed demyelination and axonal loss, suggesting an immune attack and ischemia.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Neuropathy is frequently associated with paraproteinaemia, including Waldenström's macroglobulinaemia.
- The underlying mechanisms of nerve damage in these conditions are not fully understood.
Observation:
- A 69-year-old man presented with a slowly progressive, sensory-predominant neuropathy as the initial manifestation of Waldenström's macroglobulinaemia.
- Sural nerve biopsy revealed a mixed pattern of primary demyelination and axonal degeneration.
Findings:
- Immunocytochemistry identified immunoglobulin M deposits in myelin sheaths and perineurium.
- Axonal loss was concentrated in central nerve fascicles, supporting an ischemic mechanism.
- Absence of inflammatory cell infiltrates and amyloid material in the peripheral nerve.
Implications:
- Findings suggest a dual mechanism involving ischemia and direct immunological attack on myelin in Waldenström's macroglobulinaemia-associated neuropathy.
- Further research into the pathogenesis and therapeutic strategies for this neuropathy is warranted.
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