Presentation and Outcomes of Localized Immunoglobulin Light Chain Amyloidosis: 14-Year Experience of an Academic Center.

ABSTRACT: Localized light chain amyloidosis (loc-AL) is a rare disorder characterized by localized deposition of misfolded AL fibrils. There are limited data on patterns of disease presentation and long-term outcomes. In this study, we retrospectively reviewed 146 patients with loc-AL at our institution between January 1, 2010, and March 1, 2024. We excluded patients with evidence of systemic AL amyloidosis. We calculated local (PFS_L) and systemic (PFS_s) progression free survival (PFS) and overall survival (OS) using the Kaplan-Meier method. We found that loc-AL most commonly involved the respiratory (26%), gastrointestinal (17%), head and neck (17%) and genitourinary (10%) systems. Overall, 51% patients were asymptomatic at presentation, and 16% had a co-existent autoimmune di

SUBMITTER: Dima D 

PROVIDER: S-EPMC11993898 | biostudies-literature | 2025 May

REPOSITORIES: biostudies-literature