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Primary Systemic Amyloid Neuropathy

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Amyloidosis

Part of the book series: Contemporary Hematology ((CH))

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Abstract

Primary systemic amyloidosis (PSA) is a plasma cell dyscrasia. Organ damage is caused by deposition of amyloid, derived from monoclonal light chains, in tissues including nerves. PSA damages tissues and leads to combinations of neuropathy and renal, cardiac, and liver failure. The neuropathy of PSA is relatively stereotyped with damage to small and autonomic nerve fibers and superimposed carpal tunnel syndrome. The disease is progressive and mortality is high, especially when there is early involvement of vital organs. Treatment in the past was relatively ineffective. However, with the advent of peripheral blood stem cell transplantation and chemotherapy in selected patients, prolonged survival is possible if the weight of organ damage at onset of treatment is not too high. Thus, early detection is paramount.

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Author information

Authors and Affiliations

  1. Department of Neurology, George Washington University, Washington, DC, USA

    Harman P. S. Bajwa MD

  2. Departments of Neurology and Neurosurgery, School of Medicine and Health Care Sciences, The George Washington University, Washington, DC, USA

    John J. Kelly MD

Authors
  1. Harman P. S. Bajwa MD
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  2. John J. Kelly MD
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Editor information

Editors and Affiliations

  1. Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    Morie A. Gertz

  2. Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    S. Vincent Rajkumar

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Bajwa, H.P.S., Kelly, J.J. (2010). Primary Systemic Amyloid Neuropathy. In: Gertz, M., Rajkumar, S. (eds) Amyloidosis. Contemporary Hematology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-631-3_10

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  • DOI: https://doi.org/10.1007/978-1-60761-631-3_10

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  • Publisher Name: Humana Press, Totowa, NJ

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  • Online ISBN: 978-1-60761-631-3

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