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Leukoencephalopathy in Waldenström's macroglobulinemia. Immunohistochemical and electron microscopic observations
Abstract:
The clinical and pathologic features of a case of Waldenström's macroglobulinemia with leukoencephalopathy are reported. Multiple cerebral foci of demyelination, accompanied to a lesser extent by axonal degeneration, were associated with perivascular infiltrates of plasmacytoid lymphocytes and with permeation of the white matter by macroglobulins. Immunohistochemical studies demonstrated a predominance of IgM kappa within the blood plasma, in cerebral blood vessel walls, in the foci of demyelination, and within perivascular histiocytes. Electron microscopy disclosed the presence, in macrophages and pericytes, of membrane-bound cytoplasmic inclusions consisting of tubular arrays, suggestive of cryoglobulin deposits. We hypothesize that the high serum levels of macroglobulins accompanied by lymphoplasmocytic infiltrates may, either by way of viscosity-related ischemia, or by a direct toxic effect, have caused abnormal vascular permeability, infiltration of the cerebral parenchyma by paraproteins, and, ultimately, focal degeneration of the white matter.
Insights
This study details a rare case of Waldenström's macroglobulinemia causing leukoencephalopathy. High macroglobulin levels and immune cell infiltrates damaged white matter in the brain.
Area of Science:
- Neurology
- Hematology
- Immunology
Background:
- Waldenström's macroglobulinemia is a rare lymphoproliferative disorder characterized by IgM paraprotein production.
- Neurological complications are uncommon but can be severe, impacting patient prognosis.
Observation:
- A case presenting with leukoencephalopathy, characterized by demyelination and axonal degeneration in the brain.
- Cerebral lesions showed perivascular infiltration of plasmacytoid lymphocytes and macroglobulin deposition.
- Immunohistochemistry confirmed IgM kappa predominance in plasma, vessel walls, and brain lesions.
Findings:
- Electron microscopy revealed inclusions suggestive of cryoglobulin deposits in macrophages and pericytes.
- High macroglobulin serum levels and lymphoplasmocytic infiltrates were observed.
- Evidence suggests abnormal vascular permeability and paraprotein infiltration into the brain parenchyma.
Implications:
- This case highlights a potential mechanism for CNS damage in Waldenström's macroglobulinemia.
- Understanding these mechanisms may lead to targeted therapies for neurological complications.
- Further research is needed to elucidate the direct toxic effects of macroglobulins on the central nervous system.