AL kappa amyloid in a solitary extradural lymphoma. Issue 6 (June 1994)

• Record Type: Journal Article
• Authors: Vigushin, D M; Hawkins, P N; Hsuan, J J; Totty, N F; Pepys, M B
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 57:Issue 6(1994)
• Page Start: 751
• Page End: 754
• Publication Date: 1994-06
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.57.6.751
• Languages: English
• ISSNs: 0022-3050

Abstract

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.