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Original Article
Brief Report
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Volume 329:1389-1393 November 4, 1993 Number 19
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Heavy-Chain Deposition Disease
Pierre Aucouturier, Ahmed A. Khamlichi, Guy Touchard, Eve Justrabo, Michel Cogne, Bruno Chauffert, Francois Martin, and Jean-Louis Preud'homme

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Light-chain amyloidosis and light-chain deposition disease are closely related processes characterized by tissue deposition of organized (fibrillar) or nonorganized material related to monoclonal immunoglobulin light chains. Both conditions can occur in patients with immunoproliferative disorders. The clinical manifestations of light-chain deposition disease result from multivisceral involvement and are heterogeneous. Rapidly progressive renal 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 findings may be caused by structural abnormalities or unusual behavior of deposited light chains. In particular, pathogenic light chains are 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.

References


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