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Dialysis-Associated Amyloidosis

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Amyloid and Related Disorders

Part of the book series: Current Clinical Pathology ((CCPATH))

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Abstract

Dialysis-associated amyloidosis resulting from the accumulation of beta-2 microglobulin (β2m) is an important long-term complication in patients with chronic kidney failure on long-term dialysis. Bone and periarticular tissue are the most common sites of β2m deposition. Typical clinical manifestations include the carpal tunnel syndrome, chronic arthropathy, spondyloarthropathies, subchondral bone cysts, and fractures. Although the pathogenesis of dialysis-associated amyloidosis has not been fully elucidated, there are several relevant molecular pathways that have been identified. Histopathological examination is the gold standard for diagnosing β2m-related amyloidosis. The diagnosis is made on the basis of positive Congo red staining with typical green-yellow birefringence under polarized light, coupled with positive anti-β2m antibody immunostaining. The β2m amyloid deposition starts early in the course of the disease, long before the development of symptoms. Therefore, early detection especially in high-risk patients may be important to prevent or delay disease progression before the development of debilitating complications. Novel dialysis modalities to enhance β2m removal and ultrapure dialysate to reduce β2m synthesis from chronic inflammation are treatment strategies for dialysis-related amyloidosis. However, successful kidney transplantation remains the only potential curative treatment of choice.

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Author information

Authors and Affiliations

  1. Division of Nephrology, Department of Medicine, St. Elizabeth’s Medical Center, Tufts University School of Medicine, 736 Cambridge Street, Boston, MA, 02135, USA

    Paweena Susantitaphong M.D. & Bertrand L. Jaber M.D., M.S.

  2. Department of Medicine, Division of Nephrology, University of Pennsylvania Health System, Philadelphia, PA, USA

    Laura M. Dember M.D.

Authors
  1. Paweena Susantitaphong M.D.
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  2. Laura M. Dember M.D.
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  3. Bertrand L. Jaber M.D., M.S.
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Corresponding author

Correspondence to Bertrand L. Jaber M.D., M.S. .

Editor information

Editors and Affiliations

  1. Bldg#110, Rm#2242, Department of Pathology, Loyola University Medical Center, S. First Avenue 2160, Maywood, 60153, Illinois, USA

    Maria M. Picken MD, PhD, FASN

  2. , Department of Laboratory Medicine and Pa, Mayo Clinic, First Street SW 200, Rochester, 55905, USA

    Ahmet Dogan, M.D., Ph.D.

  3. , Department of Renal Pathology, Bostwick Laboratories, Lake Ellenor Dr. 8001, Orlando, 32809, Florida, USA

    Guillermo A. Herrera, M.D.

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Susantitaphong, P., Dember, L.M., Jaber, B.L. (2012). Dialysis-Associated Amyloidosis. In: Picken MD, PhD, FASN, M., Dogan, M.D., Ph.D., A., Herrera, M.D., G. (eds) Amyloid and Related Disorders. Current Clinical Pathology. Humana Press. https://doi.org/10.1007/978-1-60761-389-3_5

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  • DOI: https://doi.org/10.1007/978-1-60761-389-3_5

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