Sex-related differences in clinical presentation and all-cause mortality in patients with cardiac transthyretin amyloidosis and light chain amyloidosis

2022 ◽  
Author(s):  
Mattia Zampieri ◽  
Alessia Argirò ◽  
Marco Allinovi ◽  
Luigi Tassetti ◽  
Chiara Zocchi ◽  
...  
Keyword(s):  
Light Chain ◽  
Clinical Presentation ◽  
Transthyretin Amyloidosis ◽  
Light Chain Amyloidosis ◽  
All Cause Mortality

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.

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

Abstract 17246: Sensitivity and Specificity of Relative Sparing of Apical Longitudinal Strain for Detection of Systemic Light-Chain Amyloidosis versus Transthyretin Amyloidosis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Michael Jiang ◽  
Julia M Simkowski ◽  
Nadia El Hangouche ◽  
Jeesoo Lee ◽  
Milica Marion ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Sensitivity And Specificity ◽  
Light Chain ◽  
Longitudinal Strain ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Control Group ◽  
Transthyretin Amyloidosis ◽  
Light Chain Amyloidosis ◽  
Significant Difference

Introduction: Relative apical sparing of longitudinal strain (RALS, the ratio of apical strain vs the rest of the heart) on echocardiography has been found to have high sensitivity and specificity for differentiating cardiac amyloidosis (CA) from other causes of left ventricular hypertrophy. Previous studies have shown no significant difference between amyloid subtypes, systemic light-chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) Hypothesis: There will be a significant difference in sensitivity and specificity of RALS to detect CA across amyloid subtypes. Methods: A cohort of patients with either AL or ATTR amyloid was identified, with a control cohort of patients with left ventricular hypertrophy (LVH) of other etiologies. Speckle tracking echocardiography was performed on EchoPAC (GE Medical Systems) software to obtain values of basal, mid, and apical longitudinal strain for each patient; relative apical strain was then calculated. Results: The TTR group (n=22) was older (66.4±7.9, 76.6±11.6, p=0.001) and more likely to be female (p=0.009) than the AL group (n=30), both groups had similar rates of hypertension, diabetes mellitus, and end stage renal disease. Echocardiographic markers of diastolic function were decreased in both groups; the AL group had decreased left ventricle end diastolic volume (60.9±25.5, 94.9±50.2, p=0.012) and mean wall thickness (1.4±0.3, 1.6±0.4 p=0.017). ROC analysis using a RALS cutoff of 2 to differentiate AL and ATTR from the LVH control group revealed similar specificity (AL 85%, ATTR 85%) and sensitivity (AL 40%, ATTR 50%). Difference in area-under-curve (AUC) was not significant (p=0.2) (figure). Conclusions: ATTR and AL amyloid have similar specificity, but ATTR has a trend towards improved sensitivity over AL for detection of CA using RALS with the previously validated threshold of 2. This might become significant with a larger sample, work that is currently on-going..

scholarly journals Differential Myocyte Responses in Patients with Cardiac Transthyretin Amyloidosis and Light-Chain Amyloidosis: A Cardiac MR Imaging Study

Radiology ◽  
2015 ◽  
Vol 277 (2) ◽  
pp. 388-397 ◽  
Author(s):  
Marianna Fontana ◽  
Sanjay M. Banypersad ◽  
Thomas A. Treibel ◽  
Amna Abdel-Gadir ◽  
Viviana Maestrini ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Light Chain ◽  
Imaging Study ◽  
Transthyretin Amyloidosis ◽  
Cardiac Mr ◽  
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
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