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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1994 Jun;57(6):751–754. doi: 10.1136/jnnp.57.6.751

AL kappa amyloid in a solitary extradural lymphoma.

D M Vigushin 1, P N Hawkins 1, J J Hsuan 1, N F Totty 1, M B Pepys 1
PMCID: PMC1072986  PMID: 8006662

Abstract

A 68 year old man with a 10 year history of apparently benign IgM kappa paraproteinaemia presented with dysarthria, left hemiparesis, and a sensory peripheral neuropathy. A calcified right temporoparietal extradural mass was shown by scintigraphy with 123I-serum amyloid P component to contain amyloid. There were no extracranial amyloid deposits. Clinical improvement followed craniotomy and partial resection of tissue which consisted of amyloid and a mixed mononuclear cell infiltrate. The amyloid fibrils consisted of the framework 1 region of the variable domain of monoclonal kappa IV immunoglobulin light chains. There was a prominent B-cell clonal immunoglobulin gene rearrangement in the tumour tissue, supporting a diagnosis of lymphoplasmacytic lymphoma, but no sign of systemic lymphoma. Neurological state, tumour volume, and quantity of amyloid have remained static for two years after treatment with chlorambucil.

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Selected References

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