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The Challenge of Systemic Immunoglobulin Light-Chain Amyloidosis (AL)

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Protein Aggregation and Fibrillogenesis in Cerebral and Systemic Amyloid Disease

Part of the book series: Subcellular Biochemistry ((SCBI,volume 65))

Abstract

The cardiac involvement and associated mortality that occur in systemic AL amyloidosis remain among the most challenging aspects of the systemic amyloid-related diseases. Monoclonal immunoglobulin light chains produced by a clone of plasma cells are usually the cause of symptoms and organ dysfunction via both poorly understood toxic effects of misfolded species and accumulation of interstitial amyloid fibrils in key viscera. Treatment is aimed at eliminating the clonal cells in order to eliminate toxic light chain production. Recent advances in therapy have helped many patients with AL achieve complete hematologic responses and significant reversal of organ damage but these benefits do not extend to that 10–15 % who present with advanced cardiac involvement. Even with cardiac transplant followed by effective therapy such as stem cell transplant, outcomes for these patients remain promising at best.

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Abbreviations

AL:

AL amyloidosis

ARTC:

Pavia Amyloidosis Research and Treatment Center

FLC:

Free light chains

SCT:

Stem cell transplant

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  1. Amyloidosis Research and Treatment Center, Foundation “IRCCS Policlinico San Matteo”, and Department of Molecular Medicine, University of Pavia, Viale Golgi 19, 27100, Pavia, Italy

    Giovanni Palladini

  2. Tufts Medical Center, 800 Washington Street, 02111, Boston, MA, USA

    Raymond L. Comenzo

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  1. Giovanni Palladini
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  2. Raymond L. Comenzo
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Correspondence to Giovanni Palladini .

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Editors and Affiliations

  1. Inst. of Zoology, University of Mainz, Becherweg 6, Mainz, 55099, Germany

    J. Robin Harris

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© 2012 Springer Science+Business Media Dordrecht

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Palladini, G., Comenzo, R. (2012). The Challenge of Systemic Immunoglobulin Light-Chain Amyloidosis (AL). In: Harris, J. (eds) Protein Aggregation and Fibrillogenesis in Cerebral and Systemic Amyloid Disease. Subcellular Biochemistry, vol 65. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-5416-4_22

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