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AL Lambda Amyloidosis Activates Acute Liver Failure in the Absence of Plasma Cell Dyscrasia

Digestive Diseases and Sciences volume 67, pages 67–70 (2022)Cite this article

Abstract

A patient with systemic amyloidosis developed portal hypertension, acute liver failure and multiorgan dysfunction. Extensive testing was unrevealing for paraproteinemia, plasma cell dyscrasia, infectious, or inflammatory conditions. He was transferred to our institution for orthotopic liver transplant evaluation but was ultimately declined given clinical instability and dysautonomia. Post-mortem evaluation revealed extensive amyloid deposition in multiple organs determined to be AL-lambda amyloidosis.

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References

  1. Voulgarelis M, Mitroulis I, Tzioufas AG. Amyloidosis. In: Efthimiou P, ed. Springer: Cham; 2019; 297–311.

    Chapter  Google Scholar 

  2. Staron A, Kataria Y, Murray DL, Sloan JM, Sanchorawala V. Systemic AL amyloidosis with an undetectable plasma cell dyscrasia: A zebra without stripes. Am J Hematol. 2020;95:E45–E48. https://doi.org/10.1002/ajh.25685.

    Article  PubMed  Google Scholar 

  3. Benson MD, Liepnieks JJ, Kluve-Beckerman B. Hereditary systemic immunoglobulin light-chain amyloidosis. Blood. 2015;125:3281–3286. https://doi.org/10.1182/blood-2014-12-618108.

    Article  CAS  PubMed  Google Scholar 

  4. Levine RA. Amyloid disease of the liver. Correlation of clinical, functional and morphologic features in forty-seven patients. Am J Med. 1962;33:349–357. doi:https://doi.org/10.1016/0002-9343(62)90231-0

  5. Norero B, Perez-Ayuso RM, Duarte I, Ramirez P, Soza A, Arrese M et al. Portal hypertension and acute liver failure as uncommon manifestations of primary amyloidosis. Ann Hepatol. 2013;13:142–149.

    Article  Google Scholar 

  6. Real de Asua D, Costa R, Galvan JM, Filigheddu MT, Trujillo D, Cadinanos J. Systemic AA amyloidosis: epidemiology, diagnosis, and management. Clin Epidemiol. 2014;6:369–377. doi:https://doi.org/10.2147/CLEP.S39981.

  7. Muchtar E, Dispenzieri A, Magen H, Grogan M, Mauermann M, McPhail ED et al. Systemic amyloidosis from A (AA) to T (ATTR): a review. J Intern Med. 2021;289:268–292. https://doi.org/10.1111/joim.13169.

    Article  CAS  PubMed  Google Scholar 

  8. Li G, Han D, Wei S, Wang H, Chen L. Multiorgan involvement by amyloid light chain amyloidosis. J Int Med Res. 2019;47:1778–1786. https://doi.org/10.1177/0300060518814337.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Park MA, Mueller PS, Kyle RA, Larson DR, Plevak MF, Gertz MA. Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients. Medicine (Baltimore). 2003;82:291–298. https://doi.org/10.1097/01.md.0000091183.93122.c7.

    Article  PubMed  Google Scholar 

  10. Vaxman I, Gertz M. When to suspect a diagnosis of amyloidosis. Acta Haematol. 2020;143:304–311. https://doi.org/10.1159/000506617.

    Article  PubMed  Google Scholar 

  11. Wang YD, Zhao CY, Yin HZ. Primary hepatic amyloidosis: a mini literature review and five cases report. Ann Hepatol. 2012;11:721–727.

    Article  Google Scholar 

Download references

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Authors and Affiliations

  1. Departments of Medicine, University of California at Los Angeles, Los Angeles, CA, USA

    Nilofar Najafian, Andrew T. Weber, Sammy Saab & Gina Choi

  2. Departments of Pathology and Laboratory Medicine, University of California at Los Angeles, Los Angeles, CA, USA

    Jonathan E. Zuckerman & Sarah Zhang

  3. Departments of Surgery, University of California at Los Angeles, Los Angeles, CA, USA

    Sammy Saab & Gina Choi

  4. Departments of Nursing, University of California at Los Angeles, Los Angeles, CA, USA

    Sammy Saab

  5. Pfleger Liver Institute, UCLA Medical Center, 200 Medical Plaza, Suite 214, Los Angeles, CA, 90095, USA

    Sammy Saab

Authors
  1. Nilofar Najafian
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  2. Andrew T. Weber
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  3. Jonathan E. Zuckerman
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  4. Sarah Zhang
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  5. Sammy Saab
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Correspondence to Sammy Saab.

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Najafian, N., Weber, A.T., Zuckerman, J.E. et al. AL Lambda Amyloidosis Activates Acute Liver Failure in the Absence of Plasma Cell Dyscrasia. Dig Dis Sci 67, 67–70 (2022). https://doi.org/10.1007/s10620-021-07253-2

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  • DOI: https://doi.org/10.1007/s10620-021-07253-2

Keywords

  • Amyloid
  • Acute liver failure
  • Portal hypertension