The Clinical Characteristics and Prognosis of Chinese Patients with Light-Chain Amyloidosis: A Retrospective Multicenter Analysis

He, Donghua; Guan, Fangshu; Hu, Minli; Zheng, Gaofeng; He, Jingsong; Han, Xiaoyan; Yang, Yang; Pan, Hong; Wang, Gang; Zhao, Yi; Wu, Wenjun; Zhang, Cai

doi:10.1007/s12288-021-01469-y

Cited by 4 publications

(1 citation statement)

References 23 publications

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“…These light chains can then form amyloid fibrils that deposit in tissues and organs, causing progressive structural damage and organ dys-function [ 1 ]. The annual incidence of AL amyloidosis in China is approximately 10 to 14 cases per million [ 2 ]. Only 20% to 30% of patients have clinical evidence of liver involvement [ 3 , 4 ]; however, the prognosis of AL amyloidosis with liver involvement is poor, with a median survival time of 8.5 months [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Successful Treatment of Systemic Light Chain Amyloidosis with Liver Involvement using Low-Frequency Daratumumab: A Case Report

Meng,

He,

Cheng

et al. 2023

Am J Case Rep

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Patient: Male, 64-year-old Final Diagnosis: Systemic light chain amyloidosis Symptoms: Fatigue and recurrent fever Clinical Procedure: — Specialty: Hematology Objective: Rare disease Background: Systemic light chain (AL) amyloidosis is a disease characterized by the deposition of amyloid fibrils throughout tissues due to the production of misfolded immunoglobulin light chains by clonally expanded populations of CD38+ plasma cells. Some patients can have liver involvement, which typically presents with nonspecific symptoms. Daratumumab, a human CD38-targeting antibody, has shown efficacy in improving hematological parameters and organ function in patients with AL amyloidosis. Low-frequency daratumumab can reduce financial burden, but whether it is effective for patients with liver involvement has not been reported. Case Report: We present the case of a 64-year-old man admitted to our hospital with fatigue and recurrent fever. Histological analysis of a liver biopsy demonstrated AL amyloidosis. Bone marrow biopsy demonstrated the presence of abnormal plasma cells. Laboratory test results demonstrated increased levels of circulating free kappa (κ) light chains, which were also seen on blood and urine immunofixation electrophoresis. Based on these findings, AL amyloidosis of the κ light chain type with liver, cardiac, and renal involvement was diagnosed. The patient ultimately achieved hematological stringent complete response, liver remission, renal complete response, and cardiac very good partial response after 2 cycles of the low-frequency daratumumab, bortezomib, and dexamethasone regimen and 4 cycles of daratumumab and dexamethasone regimen chemotherapy. Conclusions: The case indicates that low-frequency daratumumab treatment can have efficacy in AL amyloidosis with liver involvement.

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Section: Introductionmentioning

confidence: 99%

Successful Treatment of Systemic Light Chain Amyloidosis with Liver Involvement using Low-Frequency Daratumumab: A Case Report

Meng,

He,

Cheng

et al. 2023

Am J Case Rep

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Prognostic factors in Chinese patients with immunoglobulin light chain amyloidosis: a scoping review and meta-analysis

Wu,

Wang,

Gào

et al. 2024

Annals of Medicine

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Objective This scoping review and meta-analysis aimed to map the evidence regarding prognostic factors in Chinese patients with immunoglobulin light chain (AL) amyloidosis and to identify current research gaps. Methods We searched EMBASE, PubMed, and CNKI databases from their inception to 15 September 2021. All studies investigated the association between any prognostic factor and target outcomes, including overall survival (OS), progression-free survival (PFS), and end-stage renal disease (ESRD) in Chinese patients with AL amyloidosis. Results This scoping review included 52 studies, of which 44 with 6,432 patients contributed to the multivariate prognostic analysis. Multivariate analysis identified a total of 106 factors that correlated with OS, 16 factors with PFS, and 18 factors with ESRD. Five prognostic factors were significantly associated with PFS, and 11 prognostic factors were significantly associated with ESRD. Meta-analysis was only available for prognostic factors without heterogeneous cutoff values, for which hazard ratios (HRs) and their 95% confidence intervals (CIs) were reported. Meta-analysis showed that bone marrow plasma cells (BMCs) (HR: 1.96, 95% CI: 1.21–3.19, p < 0.05) and interventricular septal thickness (IVST) (HR: 1.23, 95% CI: 1.10–1.38, p < 0.05) were independently associated with OS. Conclusion The significant prognostic factors associated with OS, PFS, and ESRD in Chinese patients with AL amyloidosis were related to plasma cell tumor load, biological characteristics, cardiac involvement, renal involvement, population characteristics, and treatment. Further studies should explore additional prognostic factors in patients with AL amyloidosis to develop prognostic models.

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Clinical characterization and outcomes of a cohort of colombian patients with AL Amyloidosis

Lacouture Fierro,

Ribero Vargas,

Sánchez Cano

et al. 2023

Colomb Med

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Background: Amyloid light chain (AL) amyloidosis is characterized by amyloid fibril deposition derived from monoclonal immunoglobulin light chains, resulting in multiorgan dysfunction. Limited data exist on the clinical features of AL amyloidosis. Objective: This study aims to describe the clinical characteristics, treatments, and outcomes in Colombian patients with AL amyloidosis. Methods: A retrospective descriptive study was conducted at three high-complexity centers in Medellín, Colombia. Adults with AL amyloidosis diagnosed between 2012 and 2022 were included. Clinical, laboratory, histological, treatment, and survival data were analyzed. Results: The study included 63 patients. Renal involvement was most prevalent (66%), followed by cardiac involvement (61%). Multiorgan involvement occurred in 61% of patients. Amyloid deposition was most commonly detected in renal biopsy (40%). Bortezomib-based therapy was used in 68%, and 23.8% received high-dose chemotherapy with autologous hematopoietic stem cell transplantation (HDCT-ASCT). Hematological response was observed in 95% of patients with available data. Cardiac and renal organ responses were 15% and 14%, respectively. Median overall survival was 45.1 months (95% CI: 22.2-63.8). In multivariate analysis, cardiac involvement was significantly associated with inferior overall survival (HR 3.27; 95% CI: 1.23-8.73; p=0.018), HDCT-ASCT had a non-significant trend towards improved overall survival (HR 0.25; 95% CI: 0.06-1.09; p=0.065). Conclusions: In this study of Colombian patients with AL amyloidosis, renal involvement was more frequent than cardiac involvement. Overall survival and multiorgan involvement were consistent with data from other regions of the world. Multivariate analysis identifiedcardiac involvement and HDCT-AHCT as possible prognostic factors.

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The Clinical Characteristics and Prognosis of Chinese Patients with Light-Chain Amyloidosis: A Retrospective Multicenter Analysis

Cited by 4 publications

References 23 publications

Successful Treatment of Systemic Light Chain Amyloidosis with Liver Involvement using Low-Frequency Daratumumab: A Case Report

Successful Treatment of Systemic Light Chain Amyloidosis with Liver Involvement using Low-Frequency Daratumumab: A Case Report

Prognostic factors in Chinese patients with immunoglobulin light chain amyloidosis: a scoping review and meta-analysis

Clinical characterization and outcomes of a cohort of colombian patients with AL Amyloidosis

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