Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis · 2025
Hepatic involvement in light chain amyloidosis: analysis of 130 patients and predictors of hepatic response and survival.
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Rees Matthew J, Toumeh Nadia, Dispenzieri Angela, Gertz Morie, Yohannan Binoy, Atallah-Yunes Suheil Albert, Kapoor Prashant, Kourelis Taxiarchis, Leung Nelson, Hayman Suzanne, Buadi Francis, Dingli David, Cook Joselle, Warsame Rahma, Binder Moritz, Gonsalves Wilson, Rajkumar S Vincent, Kumar Shaji, Muchtar Eli
Аннотация
UNLABELLED: Organ response is key to improving outcomes in light chain (AL) amyloidosis. We investigated factors associated hepatic response (HepR) in a large cohort of patients with hepatic AL amyloidosis.
METHODS: Retrospective study of newly-diagnosed AL amyloidosis patients (n = 130) with liver involvement evaluated at the Mayo Clinic between 2000-2021. Patients were eligible if they had documented liver involvement and baseline alkaline phosphatase (ALP)≥1.5x upper limit of normal (ULN). HepR was defined as >50% reduction in ALP from baseline or ALP normalization. HepRs were assessed at 6, 12, 24 month after treatment initiation and the best HepR at any time point.
RESULTS: The median baseline ALP was 2.88-fold the ULN (ALP:ULN, IQR: 2.15-4.41), and the median bilirubin was 0.7 mg/dL. HepR rates increased with time from 28% at 6 months, 36% at 12 months and 48% at 24 months. The median time to HepR was 21.5 months (95%CI = 15.4-29.5). Baseline ALP ≥ 4xULN consistently predicted HepR across all time points. Hematological response (HemR) also independently predicted HepR at 12, 24 months and best response. At best hepatic response, kappa isotype, and front-line ASCT were further independent predictors of HepR.
CONCLUSIONS: The degree of baseline ALP elevation and HemR are reliable predictors of HepR.
METHODS: Retrospective study of newly-diagnosed AL amyloidosis patients (n = 130) with liver involvement evaluated at the Mayo Clinic between 2000-2021. Patients were eligible if they had documented liver involvement and baseline alkaline phosphatase (ALP)≥1.5x upper limit of normal (ULN). HepR was defined as >50% reduction in ALP from baseline or ALP normalization. HepRs were assessed at 6, 12, 24 month after treatment initiation and the best HepR at any time point.
RESULTS: The median baseline ALP was 2.88-fold the ULN (ALP:ULN, IQR: 2.15-4.41), and the median bilirubin was 0.7 mg/dL. HepR rates increased with time from 28% at 6 months, 36% at 12 months and 48% at 24 months. The median time to HepR was 21.5 months (95%CI = 15.4-29.5). Baseline ALP ≥ 4xULN consistently predicted HepR across all time points. Hematological response (HemR) also independently predicted HepR at 12, 24 months and best response. At best hepatic response, kappa isotype, and front-line ASCT were further independent predictors of HepR.
CONCLUSIONS: The degree of baseline ALP elevation and HemR are reliable predictors of HepR.