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The clinical features and outcomes of systemic light chain amyloidosis with hepatic involvement

OBJECTIVES: To evaluate the clinical characteristics and prognostic factors of hepatic systemic light chain (AL) amyloidosis. METHODS: Eighty-eight patients diagnosed AL amyloidosis with hepatic involvement between June 2004 and January 2019 were analysed retrospectively. RESULTS: The median age of...

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Detalles Bibliográficos
Autores principales: Zhao, Liang, Ren, Guisheng, Guo, Jinzhou, Chen, Wencui, Xu, Weiwei, Huang, Xianghua
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Taylor & Francis 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9067946/
https://www.ncbi.nlm.nih.gov/pubmed/35481407
http://dx.doi.org/10.1080/07853890.2022.2069281
Descripción
Sumario:OBJECTIVES: To evaluate the clinical characteristics and prognostic factors of hepatic systemic light chain (AL) amyloidosis. METHODS: Eighty-eight patients diagnosed AL amyloidosis with hepatic involvement between June 2004 and January 2019 were analysed retrospectively. RESULTS: The median age of the patients was 55 years old, and the male to female ratio was 2.8:1.The main clinical manifestations include edema, digestive symptoms, weight loss, fatigue and ascites. Fifty-one patients received treatment, 42 patients were suitable for therapeutic efficacy evaluation and 25 (59.5%) achieved haematologic response. The median survival time was nine months, and the survival rates at one year, three years and five years were 33.0%, 11.4% and 6.8%, respectively. The risk of death was 6.6 times that of those who did not achieve haematologic response. Multivariate analysis showed that baseline NT-proBNP ≥ 1800 pg/ml and total bilirubin ≥ 34.2 umol/L were predictive of all-cause death. CONCLUSIONS: KEY MESSAGES: Systemic light chain amyloidosis with hepatic involvement is associated with poor survival but rarely has specific manifestations. The significant increase of NT-proBNP and hyperbilirubinemia indicate a poor prognosis.