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Primary Light Chain (AL) Amyloidosis

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Paraproteinemia and Related Disorders

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Abstract

Amyloidosis, a disease of protein misfolding, causes progressive end-organ dysfunction due to the deposition of insoluble amyloid fibrils. Amyloidosis is an increasingly recognized disease. AL amyloidosis remains the commonest amyloid type, but wild-type transthyretin amyloidosis is likely to overtake AL as the commonest amyloid type. Delays in diagnosis remain, and most patients still present with advanced disease. Recognizing disease manifestations is critical. Education of physicians in all specialties to understand the amyloid symptom complex, the recognition that although uncommon, amyloid is not as rare as commonly considered, is important. This would allow early consideration of this disease in the differential diagnosis. Risk stratification of amyloidosis is well evolved, and proper treatment can be chosen based on the nature and degree of organ damage. Treatment outcomes and survival have substantially improved, with over half the patients with AL likely to survive more than 5 years from diagnosis. Daratumumab (with CyBorD) has now become the first licensed treatment for AL amyloidosis. Attention to supportive care remains crucial to managing patients with multiorgan involvement in a multidisciplinary setting. Reversal of end-organ damage remains a limitation in amyloid therapy. Monoclonal antibody-based approaches to accelerate amyloid removal show promise.

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Authors and Affiliations

  1. National Amyloidosis Centre, University College London, Royal Free Campus, London, UK

    Ashutosh D. Wechalekar

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  1. Ashutosh D. Wechalekar
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Correspondence to Ashutosh D. Wechalekar .

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Editors and Affiliations

  1. Rheumatology and Clinical Immunology Unit Internal Medicine Department Faculty of Medicine, Cairo University, Cairo, Egypt

    Gaafar Ragab

  2. Department of Medicine, University of Udine, Udine, Udine, Italy

    Luca Quartuccio

  3. College of Medicine, University of Saskatchewan, Saskatoon, SK, Canada

    Hadi Goubran

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Wechalekar, A.D. (2022). Primary Light Chain (AL) Amyloidosis. In: Ragab, G., Quartuccio, L., Goubran, H. (eds) Paraproteinemia and Related Disorders. Springer, Cham. https://doi.org/10.1007/978-3-031-10131-1_9

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  • DOI: https://doi.org/10.1007/978-3-031-10131-1_9

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  • Online ISBN: 978-3-031-10131-1

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