Liver involvement as the hallmark of aggressive disease in light chain amyloidosis: Distinctive clinical features and role of light chain type in 225 patients

P. Russo, G. Palladini, A. Foli, L. Zenone Bragotti, P. Milani, M. Nuvolone, L. Obici, V. Perfetti, S. Brugnatelli, R. Invernizzi, Giampaolo Merlini

Research output: Contribution to journalArticlepeer-review

Abstract

Previous studies have linked liver involvement to a poor prognosis in immunoglobulin light chain (AL) amyloidosis. However, the reason for this dismal outcome remains unclear. We compared the clinical presentation and outcome of 225 patients with and 643 subjects without liver involvement from a series of 868 consecutive patients with AL amyloidosis diagnosed between 1986 and 2007. Patients with liver involvement and λ clones had a more severe cardiac dysfunction as assessed by biomarkers and shorter median survival (1.4 years) compared to subjects with hepatic amyloidosis and κ clones (4.8 years) and to patients without liver involvement (4.4 years). The poor outcome of patients with hepatic amyloidosis compared with subjects without liver involvement among those with λ clones was confirmed in the subgroups of patients with heart involvement, with heart failure and without heart involvement.

Original languageEnglish
Pages (from-to)92-93
Number of pages2
JournalAmyloid
Volume18
Issue numberSUPPL. 1
DOIs
Publication statusPublished - Jun 2011

ASJC Scopus subject areas

  • Internal Medicine

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