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Kappa III immunoglobulin light chain origin of localized orbital amyloidosis. 2004
Kamran Hamidi Asl, and
Juris J Liepnieks, and
William R Nunery, and
Masahide Yazaki, and
Merrill D L Benson
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis 46202-5121, USA.
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.
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