Hamidi Asl Kamran, Liepnieks Juris J, Nunery William R, Yazaki Masahide, Benson Merrill D L
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis 46202-5121, USA.
Amyloid. 2004 Sep;11(3):179-83. doi: 10.1080/1350-6120400000707.
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.
孤立性眼眶淀粉样变性是一种罕见病症，其中肌肉内沉积物导致眼球突出和眼球运动受限。先前的报告提示淀粉样原纤维起源于免疫球蛋白，但这尚未得到生物化学证实。本文报告一例最初为单侧眼眶淀粉样变性进展为双侧病变的病例。对切除的眼肌进行生物化学分析确定淀粉样原纤维源自κIII免疫球蛋白轻链。文中还讨论了发病机制和组织嗜性问题。
