AL kappa amyloid in a solitary extradural lymphoma.

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.

• Authors: D M Vigushin, P N Hawkins, J J Hsuan, N F Totty, M B Pepys
• Journal: Journal of neurology, neurosurgery, and psychiatry (J Neurol Neurosurg Psychiatry) Vol. 57 Issue 6 Pg. 751-4 (Jun 1994) ISSN: 0022-3050 [Print] England
• PMID: 8006662 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Amyloid
• Immunoglobulin Light Chains
• Immunoglobulin M
• Immunoglobulin Variable Region
• Iodine Radioisotopes
• Serum Amyloid P-Component
• amyloid protein AL
• Chlorambucil

Topics

• Aged
• Amino Acid Sequence
• Amyloid (analysis, chemistry)
• Chlorambucil (therapeutic use)
• Combined Modality Therapy
• Craniotomy
• Dysarthria (etiology)
• Gene Rearrangement, B-Lymphocyte, Light Chain
• Hemiplegia (etiology)
• Humans
• Immunoblotting
• Immunoglobulin Light Chains (genetics)
• Immunoglobulin M
• Immunoglobulin Variable Region
• Iodine Radioisotopes
• Leukocytes, Mononuclear (pathology)
• Lymphoma, B-Cell (blood, chemistry, complications, diagnosis, genetics, surgery)
• Magnetic Resonance Imaging
• Male
• Molecular Sequence Data
• Paraproteinemias (complications)
• Peripheral Nervous System Diseases (etiology)
• Prospective Studies
• Serum Amyloid P-Component
• Skull Neoplasms (blood, chemistry, complications, diagnosis, genetics, surgery)