Light-chain amyloidosis and light-chain deposition disease areclosely related processes characterized by tissue depositionof organized (fibrillar) or nonorganized material related tomonoclonal immunoglobulin light chains. Both conditions canoccur in patients with immunoproliferative disorders. The clinicalmanifestations of light-chain deposition disease result frommultivisceral involvement and are heterogeneous. Rapidly progressiverenal failure or the nephrotic syndrome is the most common manifestation,but heart failure, arrhythmia, liver involvement (peliosis),and neurologic disorders may also be present. These findingsmay be caused by structural abnormalities or unusual behaviorof deposited light chains. In particular, pathogenic light chainsare characterized by unusual . . . [Full Text of this Article]
Methods
Patients
Pathological Studies
Immunochemical Studies
Study of Messenger RNA in Patient 1
Results
Histopathological Studies
Immunochemical and Sequencing Studies of Serum and Urine
Discussion
Source Information
From the Laboratory of Immunology and Molecular Interactions (Centre National de la Recherche Scientifique Associated Research Unit 1172), University Hospital and Faculty of Sciences, Poitiers (P.A., A.A.K., M.C., J.-L.P.); the Department of Nephrology, University Hospital, Poitiers (G.T.); and the Department of Pathology (E.J.) and the Human and Experimental Digestive Cancer Unit (Institut National de la Sante et de la Recherche Medicale Unit 252) (B.C., F.M.), University Hospital, Dijon -- all in France.
Address reprint requests to Dr. Aucouturier at the Centre National de la Recherche Scientifique, Unite de Recherche Associee 1172, Centre Hospitalier Universitaire, B.P. 577, F-86021 Poitiers, France.
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