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Immunoglobulin light chain amyloidosis and the kidney

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Monoclonal Gammopathies and the Kidney

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Abstract

Primary systemic amyloidosis, also known as immunoglobulin light chain amyloidosis (AL) is a plasma cell dyscrasia. In AL a clonal population of plasma cells produces a monoclonal light chain or fragment thereof. The immunoglobulin light chain fragment forms a beta-pleated sheet protofilament that ultimately polymerizes into the complete amyloid fiibril. Amyloid fibrils are nonbiodegradable1. Accumulation of the fibril protein eventually results in organ dysfunction and death. Involvement of the kidneys is the most common clinical problem in AL and is present in one-third of patients at the time of diagnosis2. At post mortem examination most patients have evidence of amyloid deposition within the kidney3.

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Authors and Affiliations

  1. Division of Hematology and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, MN, USA

    M. A. Gertz, M. Q. Lacy & A. Dispenzieri

Authors
  1. M. A. Gertz
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  2. M. Q. Lacy
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  3. A. Dispenzieri
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Editor information

Editors and Affiliations

  1. University Hospital, Poitiers, France

    Guy Touchard

  2. Tenon Hospital, Paris, France

    Pierre Aucouturier  & Pierre Ronco  & 

  3. Necker Hospital, Paris, France

    Olivier Hermine

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© 2003 Springer Science+Business Media Dordrecht

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Gertz, M.A., Lacy, M.Q., Dispenzieri, A. (2003). Immunoglobulin light chain amyloidosis and the kidney. In: Touchard, G., Aucouturier, P., Hermine, O., Ronco, P. (eds) Monoclonal Gammopathies and the Kidney. Springer, Dordrecht. https://doi.org/10.1007/978-94-017-0191-4_18

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  • DOI: https://doi.org/10.1007/978-94-017-0191-4_18

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-90-481-6199-7

  • Online ISBN: 978-94-017-0191-4

  • eBook Packages: Springer Book Archive

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