scholarly journals The Clinical Presentation and Management of Systemic Light-Chain Amyloidosis in China

2016 ◽  
Vol 2 (1) ◽  
pp. 1-9 ◽  
Author(s):  
Xiang-Hua Huang ◽  
Zhi-Hong Liu
Keyword(s):  
Light Chain ◽  
Clinical Presentation ◽  
Light Chain Amyloidosis

Clinical presentation and outcomes in light chain amyloidosis patients with non-evaluable serum free light chains

Leukemia ◽  
2017 ◽  
Vol 32 (3) ◽  
pp. 729-735 ◽  
Author(s):  
S Sidana ◽  
N Tandon ◽  
A Dispenzieri ◽  
M A Gertz ◽  
F K Buadi ◽  
...  
Keyword(s):  
Light Chain ◽  
Clinical Presentation ◽  
Light Chains ◽  
Free Light Chains ◽  
Serum Free ◽  
Light Chain Amyloidosis ◽  
Serum Free Light Chains

Tumor cells in light-chain amyloidosis and myeloma show different transcriptional rewiring of normal plasma cell development

Blood ◽  
2021 ◽  
Author(s):  
Daniel Alameda ◽  
Ibai Goicoechea ◽  
Marco Vicari ◽  
Elena Arriazu ◽  
Alice Nevone ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Plasma Cell ◽  
Light Chain ◽  
Clinical Presentation ◽  
Light Chain Amyloidosis ◽  
New Born ◽  
Transcriptional Rewiring ◽  
Differentiation Stage ◽  
Secondary Lymphoid Organs

Although light-chain amyloidosis (AL) and multiple myeloma (MM) are characterized by tumor plasma cell (PC) expansion in bone marrow (BM), their clinical presentation differs. Previous attempts to identify unique pathogenic mechanisms behind such differences were unsuccessful, but there are no studies investigating the differentiation stage of tumor PCs in patients with AL and MM. We sought to define a transcriptional atlas of normal PC development (n=11) in secondary lymphoid organs (SLO), peripheral blood (PB) and BM for comparison with the transcriptional programs (TPs) of tumor PCs in AL (n=37), MM (n=46) and MGUS (n=6). Based on bulk and single-cell RNAseq, we observed thirteen TPs during transition of normal PCs throughout SLO, PB and BM; that CD39 outperforms CD19 to discriminate new-born from long-lived BM-PCs; that tumor PCs expressed the most advantageous TPs of normal PC differentiation; that AL shares greater similarity to SLO-PCs whereas MM is transcriptionally closer to PB-PCs and new-born BM-PCs; that AL and MM patients enriched in immature TPs had inferior survival; and that TPs related with protein N-linked glycosylation are upregulated in AL. Collectively, we provide a novel resource to understand normal PC development and the transcriptional reorganization of AL and other monoclonal gammopathies.

Clinical presentation and prognosis of light-chain amyloidosis patients with unmeasurable free light-chain levels

2018 ◽  
Vol 97 (12) ◽  
pp. 2465-2470 ◽  
Author(s):  
Yu Qiu ◽  
Cong-li Zhang ◽  
Kai-ni Shen ◽  
Wei Su ◽  
Jun Feng ◽  
...  
Keyword(s):  
Light Chain ◽  
Clinical Presentation ◽  
Free Light Chain ◽  
Light Chain Amyloidosis

scholarly journals 110 Sex-related differences in clinical presentation and outcome of patients with cardiac transthyretin amyloidosis and light chain amyloidosis

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Mattia Zampieri ◽  
Alessia Argirò ◽  
Marco Allinovi ◽  
Luigi Tassetti ◽  
Chiara Zocchi ◽  
...  
Keyword(s):  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Clinical Presentation ◽  
Nyha Class ◽  
Interventricular Septum ◽  
University Hospital ◽  
Wild Type ◽  
Transthyretin Amyloidosis ◽  
Light Chain Amyloidosis ◽  
Different Types

Abstract Aims We aimed to ascertain whether sex-related differences are relevant to the pathophysiology, presentation, and outcomes in different types of cardiac amyloidosis—a field still poorly investigated. Medical files from consecutive patients diagnosed with cardiac amyloidosis between 2000 and 2020, at Careggi University Hospital, were retrospectively evaluated. Methods and results Over this period, 259 patients (12% females) were diagnosed with wild type transthyretin amyloidosis (wtATTR), 52 (25% females) with hereditary transthyretin amyloidosis (hATTR) and 143 (47% females) with light chain amyloidosis (AL). Wt-ATTR women, compared to men, were significantly older at the time of diagnosis and showed higher National Amyloidosis Centre score, thicker normalized interventricular septum, higher diastolic dysfunction and worse right ventricular function. Females with hATTR and AL had lower normalized cardiac mass compared to men. Otherwise, bio-humoural parameters, NYHA class, and ECG characteristics were similar. No differences in outcome were observed with regard to sex. Conclusions In conclusion, we did not observe major differences in clinical expression related to sex in different types of cardiac amyloidosis: specifically, outcome was not affected. Nevertheless, women with wtATTR showed a worse profile at diagnosis and evidence suggesting a later recognition of disease compared to men, highlighting the need for a higher index of suspicion in female patients.

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