AL kappa amyloid in a solitary extradural lymphoma.
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.
Vigushin
D M
DM
Immunological Medicine Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London, UK.
Hawkins
P N
PN
Hsuan
J J
JJ
Totty
N F
NF
Pepys
M B
MB
D000368:Aged; D000595:Amino Acid Sequence; D000682:Amyloid / Q000032:analysis* / Q000737:chemistry; D002699:Chlorambucil / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003399:Craniotomy; D004401:Dysarthria / Q000209:etiology; D015328:Gene Rearrangement, B-Lymphocyte, Light Chain; D006429:Hemiplegia / Q000209:etiology; D006801:Humans; D015151:Immunoblotting; D007147:Immunoglobulin Light Chains / Q000235:genetics*; D007075:Immunoglobulin M; D007135:Immunoglobulin Variable Region; D007457:Iodine Radioisotopes; D007963:Leukocytes, Mononuclear / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000097:blood / Q000737:chemistry* / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D008969:Molecular Sequence Data; D010265:Paraproteinemias / Q000150:complications*; D010523:Peripheral Nervous System Diseases / Q000209:etiology; D011446:Prospective Studies; D000683:Serum Amyloid P-Component; D012888:Skull Neoplasms / Q000097:blood / Q000737:chemistry* / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000601:surgery
D000682:Amyloid; D007147:Immunoglobulin Light Chains; D007075:Immunoglobulin M; D007135:Immunoglobulin Variable Region; D007457:Iodine Radioisotopes; D000683:Serum Amyloid P-Component; C035222:amyloid protein AL; D002699:Chlorambucil