Kappa III immunoglobulin light chain origin of localized orbital amyloidosis.
Isolated orbital amyloidosis is a rare condition in which intra-muscular deposits result in proptosis and restriction of eye movement. Previous reports have suggested an immunoglobulin origin of the amyloid fibrils, but this has not been proven biochemically. A case is presented in which initial unilateral orbital amyloidosis progressed to bilateral disease. Biochemical analysis of resected ocular muscle determined that the amyloid fibrils were derived from a kappa III immunoglobulin light chain. Questions of pathogenesis and tissue tropism are considered.
Hamidi Asl
Kamran
K
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis 46202-5121, USA.
Liepnieks
Juris J
JJ
Nunery
William R
WR
Yazaki
Masahide
M
Benson
Merrill D L
MD
D000328:Adult; D000686:Amyloidosis / Q000000981:diagnostic imaging / Q000473:pathology*; D005094:Exophthalmos / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D006801:Humans; D007145:Immunoglobulin kappa-Chains; D009801:Oculomotor Muscles / Q000000981:diagnostic imaging / Q000473:pathology*; D009916:Orbital Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D014057:Tomography, X-Ray Computed
D007145:Immunoglobulin kappa-Chains