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[Peripheral neuropathies i- Waldenstrom's disease. Histological and ultrastructural studies of 5 cases]
Abstract:
In four cases it was the existence of peripheral neuropathy which led to the discovery of macroglobulinemia within an interval from 1 to 11 years. The treatment of the disease also markedly improved the peripheral neuropathy in three cases. Two patients died. Two of the biopsies of the peripheral nerve showed infiltration by atypical lymphocytes. Ultrastructural changes in the five cases were variable. Marked axonal involvement in the two cases with cellular infiltration. In one of these two cases it was combined with segmental demyelinization. This lesion was clearly predominant in the three other cases. In one of them demyelinization occurred in a very peculiar way with dilation of the internal part of the mesaxone going on in the most peripheral layers of the myelin. Discussion of the mechanisms of the involvement of the peripheral nervous parenchyma in Waldenström's disease. Cellular infiltrations, amorphous deposits, changes in the capillaries and macroglobuline may occur.
Insights
Peripheral neuropathy can be an early indicator of macroglobulinemia. Treating Waldenström's disease often improves nerve damage, highlighting a crucial link between these conditions.
Area of Science:
- Neurology
- Hematology
Background:
- Peripheral neuropathy can precede the diagnosis of macroglobulinemia by several years.
- Waldenström's disease is a rare lymphoproliferative disorder affecting B lymphocytes.
Observation:
- Four cases presented with peripheral neuropathy leading to macroglobulinemia diagnosis.
- Peripheral nerve biopsies revealed atypical lymphocyte infiltration in two cases.
- Ultrastructural nerve changes varied, including axonal damage and peculiar demyelination patterns.
Findings:
- Treatment for macroglobulinemia improved peripheral neuropathy in three out of four patients.
- Axonal involvement was prominent in cases with cellular infiltration.
- Segmental demyelination was the predominant lesion in other cases, with unique myelin alterations observed.
Implications:
- Peripheral neuropathy warrants investigation for underlying macroglobulinemia.
- Understanding nerve involvement mechanisms in Waldenström's disease is crucial for patient management.
- Potential mechanisms include cellular infiltration, deposits, capillary changes, and direct macroglobulin effects.