Literature DB >> 34655012

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

Nilofar Najafian1, Andrew T Weber1, Jonathan E Zuckerman2, Sarah Zhang2, Sammy Saab3,4,5,6, Gina Choi1,7.   

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.

Entities:  

Keywords:  Acute liver failure; Amyloid; Portal hypertension

Mesh:

Substances:

Year:  2021        PMID: 34655012     DOI: 10.1007/s10620-021-07253-2

Source DB:  PubMed          Journal:  Dig Dis Sci        ISSN: 0163-2116            Impact factor:   3.199


  6 in total

1.  Hereditary systemic immunoglobulin light-chain amyloidosis.

Authors:  Merrill D Benson; Juris J Liepnieks; Barbara Kluve-Beckerman
Journal:  Blood       Date:  2015-04-09       Impact factor: 22.113

Review 2.  When to Suspect a Diagnosis of Amyloidosis.

Authors:  Iuliana Vaxman; Morie Gertz
Journal:  Acta Haematol       Date:  2020-04-27       Impact factor: 2.195

3.  Systemic AL amyloidosis with an undetectable plasma cell dyscrasia: A zebra without stripes.

Authors:  Andrew Staron; Yachana Kataria; David L Murray; J Mark Sloan; Vaishali Sanchorawala
Journal:  Am J Hematol       Date:  2019-11-26       Impact factor: 10.047

Review 4.  Systemic amyloidosis from A (AA) to T (ATTR): a review.

Authors:  E Muchtar; A Dispenzieri; H Magen; M Grogan; M Mauermann; E D McPhail; P J Kurtin; N Leung; F K Buadi; D Dingli; S K Kumar; M A Gertz
Journal:  J Intern Med       Date:  2020-09-14       Impact factor: 8.989

5.  Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients.

Authors:  Miguel A Park; Paul S Mueller; Robert A Kyle; Dirk R Larson; Matthew F Plevak; Morie A Gertz
Journal:  Medicine (Baltimore)       Date:  2003-09       Impact factor: 1.889

6.  Multiorgan involvement by amyloid light chain amyloidosis.

Authors:  Guoliang Li; Dan Han; Suhua Wei; Huaiyu Wang; Limei Chen
Journal:  J Int Med Res       Date:  2019-02-25       Impact factor: 1.671

  6 in total

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