Hepatic multimodal phenotyping in AL amyloidosis with cardiac involvement: the D-Amy-LIPHE study

Résumé fourni par la source

In light-chain (AL) amyloidosis, liver involvement is defined using arbitrary criteria such as hepatomegaly or elevated Alkaline Phosphatase (ALP). We aimed to determine its prevalence and to characterize its phenotype and prognostic impact. This study included 200 patients with AL amyloidosis. Liver involvement was defined by MRI/CT hepatomegaly and/or elevated ALP. Liver phenotyping included early-phase 99mTc-HMDP bone scintigraphy (BS), and histological analysis (n = 12). Predictors of liver involvement were identified using logistic regression. Unsupervised clustering analysis was performed to identify distinct patterns of liver involvement. Liver involvement was identified in 71 patients (35%) and was independently associated with digestive and renal amyloidosis, liver uptake and increased vena cava diameter. Clustering analysis identified three phenotypes: a cardiac cluster, characterized by severe cardiac failure and congestive hepatopathy associated with elevated bilirubin; a hepatic cluster, marked by multivisceral amyloidosis, elevated γGT and ALP, in which liver involvement reflected amyloid deposition; an intermediate cluster with milder abnormalities and better prognosis. Histological findings supported this. Sixty-month survival was 32%, 44% and 54% in the cardiac, hepatic and intermediate clusters, respectively. Liver involvement in AL amyloidosis is due to both amyloid deposition and cardiac congestion. Clustering analysis revealed distinct liver patterns with different prognoses.

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