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

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

doi:10.1007/s10620-021-07253-2

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

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

Authors

Nilofar Najafian¹, Andrew T Weber¹, Jonathan E Zuckerman², Sarah Zhang², Sammy Saab^{3

4

5

6}, Gina Choi^{1

7}

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. SSaab@mednet.ucla.edu.
⁴ Departments of Surgery, University of California at Los Angeles, Los Angeles, CA, USA. SSaab@mednet.ucla.edu.
⁵ Departments of Nursing, University of California at Los Angeles, Los Angeles, CA, USA. SSaab@mednet.ucla.edu.
⁶ Pfleger Liver Institute, UCLA Medical Center, 200 Medical Plaza, Suite 214, Los Angeles, CA, 90095, USA. SSaab@mednet.ucla.edu.
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.

Keywords: Acute liver failure; Amyloid; Portal hypertension.

Publication types

Case Reports

MeSH terms

Amyloidosis, Familial* / complications
Amyloidosis, Familial* / diagnosis
Amyloidosis, Familial* / physiopathology
Ascites* / diagnosis
Ascites* / etiology
Ascites* / therapy
Clinical Deterioration
Fatal Outcome
Hepatic Encephalopathy / diagnosis
Hepatic Encephalopathy / etiology
Hepatic Encephalopathy / therapy
Humans
Image-Guided Biopsy / methods
Immunoglobulin lambda-Chains / isolation & purification
Intestinal Obstruction / diagnosis
Intestinal Obstruction / etiology
Intestinal Obstruction / therapy
Liver Failure, Acute* / diagnosis
Liver Failure, Acute* / etiology
Liver Failure, Acute* / therapy
Liver Function Tests / methods
Liver* / diagnostic imaging
Liver* / pathology
Male
Middle Aged
Paracentesis / methods
Plaque, Amyloid* / diagnostic imaging
Plaque, Amyloid* / metabolism
Plaque, Amyloid* / pathology

Substances

Immunoglobulin lambda-Chains