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Dig Dis Sci. 2022 Jan;67(1):67-70. doi: 10.1007/s10620-021-07253-2. Epub 2021 Oct 15.

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

Digestive diseases and sciences

Nilofar Najafian, Andrew T Weber, Jonathan E Zuckerman, Sarah Zhang, Sammy Saab, Gina Choi

Affiliations

  1. Departments of Medicine, University of California at Los Angeles, Los Angeles, CA, USA.
  2. Departments of Pathology and Laboratory Medicine, University of California at Los Angeles, Los Angeles, CA, USA.
  3. Departments of Medicine, University of California at Los Angeles, Los Angeles, CA, USA. [email protected].
  4. Departments of Surgery, University of California at Los Angeles, Los Angeles, CA, USA. [email protected].
  5. Departments of Nursing, University of California at Los Angeles, Los Angeles, CA, USA. [email protected].
  6. Pfleger Liver Institute, UCLA Medical Center, 200 Medical Plaza, Suite 214, Los Angeles, CA, 90095, USA. [email protected].
  7. Departments of Surgery, University of California at Los Angeles, Los Angeles, CA, USA.

PMID: 34655012 DOI: 10.1007/s10620-021-07253-2

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.

© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.

Keywords: Acute liver failure; Amyloid; Portal hypertension

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