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.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Aged
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Amino Acid Sequence
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Amyloid / analysis*
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Amyloid / chemistry
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Chlorambucil / therapeutic use
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Combined Modality Therapy
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Craniotomy
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Dysarthria / etiology
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Gene Rearrangement, B-Lymphocyte, Light Chain
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Hemiplegia / etiology
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Humans
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Immunoblotting
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Immunoglobulin Light Chains / genetics*
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Immunoglobulin M*
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Immunoglobulin Variable Region
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Iodine Radioisotopes
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Leukocytes, Mononuclear / pathology
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Lymphoma, B-Cell / blood
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Lymphoma, B-Cell / chemistry*
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Lymphoma, B-Cell / complications*
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Lymphoma, B-Cell / diagnosis
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Lymphoma, B-Cell / genetics
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Lymphoma, B-Cell / surgery
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Magnetic Resonance Imaging
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Male
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Molecular Sequence Data
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Paraproteinemias / complications*
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Peripheral Nervous System Diseases / etiology
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Prospective Studies
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Serum Amyloid P-Component
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Skull Neoplasms / blood
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Skull Neoplasms / chemistry*
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Skull Neoplasms / complications*
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Skull Neoplasms / diagnosis
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Skull Neoplasms / genetics
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Skull Neoplasms / surgery
Substances
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Amyloid
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Immunoglobulin Light Chains
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Immunoglobulin M
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Immunoglobulin Variable Region
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Iodine Radioisotopes
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Serum Amyloid P-Component
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amyloid protein AL
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Chlorambucil