Clinical characteristics and survival analysis of a large cohort of Light Chain amyloidosis: a single center real-world study

Abstract

Abstract Light chain (AL) amyloidosis is a plasma cell dyscrasia characterized by the tissue deposition of misfolded amyloid fibrils, leading to progressive organs dysfunction. We retrospectively analyzed 335 patients with AL amyloidosis in the First Hospital of Peking University from January 2011 to December 2021 to describe the real-world clinical characteristics and prognosis of the patients with AL amyloidosis in China. Patients (median age, 60 years) were diagnosed with multi-organ involvement of kidney (92.8%), heart (57.9%), liver (12.8%). 55.8% of patients were treated with chemotherapy, 63.4% of whom achieved hematologic response (≥ very good partial response). Only 18.2% of the patients received autologous stem cell transplant (ASCT), who were younger and less likely to have cardiac involvement. The median overall survival of patients with AL amyloidosis was 77.5 months. The level of brain natriuretic peptide, percentage of bone marrow plasma cell and total bilirubin were identified as independent prognostic factors for survival. Albeit the younger age and high ratio of renal involvement might contribute to the favorable prognosis of this cohort, the role of novel agents and ASCT is also discernible. This real-world research will provide a panoramic impression on the progress of AL amyloidosis in China.