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IgM Amyloidosis

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Abstract

Primary light chain amyloidosis (AL) is the most common form of systemic amyloidosis and is caused by misfolded light chains that cause proteotoxicity and rapid decline of vital organ function. Four percent are associated with an IgM monoclonal protein. Early diagnosis is vital in order to deliver effective therapy and prevent irreversible organ damage. Accurate diagnosis requires clinical skills and advanced technologies. The disease can be halted, and the function of target organs is rescued by the prompt reduction and elimination of the plasma cell clone producing the toxic light chains in the bone marrow. Heart damage is the major determinant of survival, and staging with cardiac biomarkers guides treatment. Two-thirds of patients can benefit from the treatment with improved quality of life and extended survival. Future efforts should be directed at anticipating the diagnosis, improving the tolerability and efficacy of anti-plasma cell therapy, accelerating recovery of organ function via resorption of amyloid deposits, and developing novel approaches to counter light chain proteotoxicity.

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Author information

Authors and Affiliations

  1. Division of Hematology, Mayo Clinic, 200 First Street, SW, Rochester, MN, USA

    Morie A. Gertz MD, MACP, Angela Dispenzieri MD & Francis K. Buadi MD

  2. Department of Medicine, College of Medicine, Mayo Distinguished Clinician, Rochester, MN, 55905, USA

    Morie A. Gertz MD, MACP

  3. Department of Hematology, Mayo Clinic, Jacksonville, FL, USA

    Taimur Sher MD

Authors
  1. Morie A. Gertz MD, MACP
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  2. Taimur Sher MD
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  3. Angela Dispenzieri MD
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  4. Francis K. Buadi MD
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Corresponding author

Correspondence to Morie A. Gertz MD, MACP .

Editor information

Editors and Affiliations

  1. Haematology, Hôpital Pitié-Salpêtriêre, Paris, France

    Véronique Leblond

  2. Dana Farber Cancer Institute, Boston, Massachusetts, USA

    Steve Treon

  3. School of Medicine, University of Athens, Athens, Greece

    Meletios Dimoploulos

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Gertz, M.A., Sher, T., Dispenzieri, A., Buadi, F.K. (2017). IgM Amyloidosis. In: Leblond, V., Treon, S., Dimoploulos, M. (eds) Waldenström’s Macroglobulinemia. Springer, Cham. https://doi.org/10.1007/978-3-319-22584-5_14

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  • DOI: https://doi.org/10.1007/978-3-319-22584-5_14

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