scholarly journals Nomogram Model for Dynamic and Individual Prediction of Cardiac Response and Survival for Light Chain Amyloidosis in 737 Patients With Cardiac Involvement

2021 ◽  
Vol 11 ◽  
Author(s):  
Yang Li ◽  
Yanze Cao ◽  
Mingxin Zheng ◽  
Jiaqi Hu ◽  
Wei Yan ◽  
...  
Keyword(s):  
Light Chain ◽  
Cardiac Involvement ◽  
Proportional Hazards ◽  
Dynamic Assessment ◽  
Cox Proportional Hazards ◽  
Assessment System ◽  
Assessment Model ◽  
Cardiac Response ◽  
Related Factors ◽  
Light Chain Amyloidosis

ObjectiveLight chain amyloidosis (AL) with cardiac involvement is associated with poor prognosis. The existing prognostic assessment system does not consider treatment-related factors, and there is currently no effective system for predicting the response. The purpose of this study was to build an individualized, dynamic assessment model for cardiac response and overall survival (OS) for AL patients with cardiac involvement.MethodsThe records of 737 AL patients with cardiac involvement were collected through cooperation with 18 hospitals in the Chinese Registration Network for Light-chain Amyloidosis (CRENLA). We used univariate and multivariate analyses to evaluate the prognostic factors for OS and cardiac response. Then, two nomogram models were developed to predict OS and cardiac response in AL patients with cardiac involvement.ResultsA nomogram including four independent factors from the multivariate Cox proportional hazards analysis—Mayo staging, courses of treatment, hematologic response, and cardiac response—was constructed to calculate the possibility of achieving survival by adding all the points associated with four variables. The higher the score, the more likely death would occur. The other nomogram model included the courses of treatment, hematological response, and different treatment regimens, and was correlated with cardiac response. The higher the score, the more likely a cardiac response would occur.ConclusionIn conclusion, based on the large Chinese cohort of patients with AL and cardiac involvement, we identified nomogram models to predict cardiac response and OS. These models are more individualized and dynamic, and therefore, they have important clinical application value.

The Utility of High Sensitivity Cardiac Troponin Among Patients with Immunoglobulin Light Chain Amyloidosis

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2887-2887 ◽  
Author(s):  
Angela Dispenzieri ◽  
Morie A Gertz ◽  
Amy K Saenger ◽  
Martha Grogan ◽  
Shaji Kumar ◽  
...  
Keyword(s):  
Light Chain ◽  
Cardiac Involvement ◽  
High Sensitivity ◽  
Staging System ◽  
Cardiac Response ◽  
Al Amyloidosis ◽  
Immunoglobulin Light Chain ◽  
Light Chain Amyloidosis ◽  
Staging Systems ◽  
Immunoglobulin Light Chain Amyloidosis

Abstract Abstract 2887 Introduction: Cardiac involvement is the major cause of death in patients with immunoglobulin light chain amyloidosis (AL). Detection of cardiac involvement and risk stratification has been facilitated by cardiac biomarkers like troponin T (cTnT) and N-terminal brain natriuretic peptide (NT-proBNP). A novel high sensitivity cTnT (hs-cTnT) assay has been developed, and we evaluated its diagnostic use with three questions in mind: 1) How do the cTnT and hs-cTnT perform in the AL amyloid staging system? 2) Does higher sensitivity add significant additional value in terms of prognosticating outcomes for patients with AL amyloidosis? 3) Can the current AL amyloidosis staging system be further improved upon? Methods: Stored serum samples (-20°C) from 224 pts with AL were analyzed for concentrations of hsTnT, TnT, and NT-proBNP on the E170 Modular analyzer (Roche Diagnostics, Penzberg, Germany). 99th percentile reference limits were <0.014 and <0.010 mcg/L for hsTnT and TnT, respectively. Results: Median values for hsTnT, TnT, and NT-proBNP were 38 ng/L (range 0–075.4), 0.017 mcg/L (<0.0–0.904), and 1230 ng/L (0–32, 226), respectively. The correlation coefficient between hsTnT and TnT was 0.972. Those classified by echocardiographic parameters as having (n=143) or not having (n=81) cardiac involvement had TnT concentrations of 0.04 and 0.01 mcg/L and hsTnT levels of 52.2 and 15.6 ng/L, respectively. The direct numeric result from the hs-cTnT result CANNOT merely be substituted for a cTnT result in the Mayo AL staging system since 14% of patients would be misclassified. The performance of the receiver operation curve derived hs-cTnT cut-point of 54 ng/L is a slight improvement over the direct substitution of 35 ng/L if replacement of one assay for another is required. An alternate staging option using hs-cTnT alone—using the two thresholds14 ng/L and 54 ng/L (figure)—performs as well as either the original Mayo AL staging system or a derivative system incorporating hs-cTnT with respective relative risks of death (95%CI) of 3.6 (2.3, 5.7), 3.8 (2.5, 5.9), and 3.3 (2.2, .50). On multivariate analysis, our newly described alternate 3 level, hs-cTnT alone staging system is independent of other factors including period of diagnosis, type of therapy, and NT-proBNP value, the last of which dropped out of the model. Alternate models using NT-proBNP and cTnT were explored, but none performed better than the original staging system or the new hs-cTnT system. Conclusion: The direct numeric result from the hs-cTnT result cannot merely be substituted for a cTnT result in the Mayo amyloid staging system. Consideration could be made for AL staging systems using hs-cTnT alone and relegating the NT-proBNP for measuring cardiac response. Disclosures: Jaffe: Roche: Consultancy.

scholarly journals Risk of schizophrenia, schizoaffective, and bipolar disorders by migrant status, region of origin, and age-at-migration: a national cohort study of 1.8 million people

2018 ◽  
Vol 49 (14) ◽  
pp. 2354-2363 ◽  
Author(s):  
Jennifer Dykxhoorn ◽  
Anna-Clara Hollander ◽  
Glyn Lewis ◽  
Cecelia Magnusson ◽  
Christina Dalman ◽  
...  
Keyword(s):  
Psychotic Disorders ◽  
Proportional Hazards ◽  
Bipolar Disorders ◽  
Cox Proportional Hazards ◽  
Migrant Status ◽  
Region Of Origin ◽  
Related Factors ◽  
Increased Risk ◽  
Age At Migration ◽  
National Cohort

AbstractBackgroundWe assessed whether the risk of various psychotic disorders and non-psychotic bipolar disorder (including mania) varied by migrant status, a region of origin, or age-at-migration, hypothesizing that risk would only be elevated for psychotic disorders.MethodsWe established a prospective cohort of 1 796 257 Swedish residents born between 1982 and 1996, followed from their 15th birthday, or immigration to Sweden after age 15, until diagnosis, emigration, death, or end of 2011. Cox proportional hazards models were used to model hazard ratios by migration-related factors, adjusted for covariates.ResultsAll psychotic disorders were elevated among migrants and their children compared with Swedish-born individuals, including schizophrenia and schizoaffective disorder (adjusted hazard ratio [aHR]migrants: 2.20, 95% CI 1.96–2.47; aHRchildren : 2.00, 95% CI 1.79–2.25), affective psychotic disorders (aHRmigrant1.42, 95% CI 1.25–1.63; aHRchildren: 1.22 95% CI 1.07–1.40), and other non-affective psychotic disorders (aHRmigrant: 1.97, 95% CI 1.81–2.14; aHRchildren: 1.68, 95% CI 1.54–1.83). For all psychotic disorders, risks were generally highest in migrants from Africa (i.e. aHRschizophrenia: 5.24, 95% CI 4.26–6.45) and elevated at most ages-of-migration. By contrast, risk of non-psychotic bipolar disorders was lower for migrants (aHR: 0.58, 95% CI 0.52–0.64) overall, and across all ages-of-migration except infancy (aHR: 1.20; 95% CI 1.01–1.42), while risk for their children was similar to the Swedish-born population (aHR: 1.00, 95% CI 0.93–1.08).ConclusionsIncreased risk of psychiatric disorders associated with migration and minority status may be specific to psychotic disorders, with exact risk dependent on the region of origin.

Usefulness of Diastolic Myocardial Stiffness Assessed by Diastolic Wall Strain in Assessing Cardiac Involvement in Light-chain Amyloidosis

2014 ◽  
Vol 20 (10) ◽  
pp. S166
Author(s):  
Masayoshi Yamamoto ◽  
Yoshihiro Seo ◽  
Naoto Kawamatsu ◽  
Noriaki Sugano ◽  
Akiko Atsumi ◽  
...  
Keyword(s):  
Light Chain ◽  
Cardiac Involvement ◽  
Myocardial Stiffness ◽  
Light Chain Amyloidosis ◽  
Diastolic Wall Strain

scholarly journals Diagnostic approach to light-chain cardiac amyloidosis and its differential diagnosis

2018 ◽  
Vol 49 (1) ◽  
pp. 9-14
Author(s):  
Monika Adamska ◽  
Anna Komosa ◽  
Tatiana Mularek ◽  
Joanna Rupa-Matysek ◽  
Lidia Gil
Keyword(s):  
Differential Diagnosis ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Diagnosis And Treatment ◽  
Diagnostic Approach ◽  
Al Amyloidosis ◽  
Delay In Diagnosis ◽  
Light Chain Amyloidosis ◽  
Novel Approaches

AbstractCardiac amyloidosis is a rare and often-misdiagnosed disorder. Among other forms of deposits affecting the heart, immunoglobulin-derived light-chain amyloidosis (AL amyloidosis) is the most serious form of the disease. Delay in diagnosis and treatment may have a major impact on the prognosis and outcomes of patients. This review focuses on the presentation of the disorder and current novel approaches to the diagnosis of cardiac involvement in AL amyloidosis.

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

2019 ◽  
Author(s):  
Donghua He ◽  
Fangshu Guan ◽  
Minli Hu ◽  
Gaofeng Zheng ◽  
Pan Hong ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Light Chain ◽  
Clinical Characteristics ◽  
Cardiac Involvement ◽  
Renal Involvement ◽  
Chinese Patients ◽  
Response To Treatment ◽  
Light Chain Amyloidosis ◽  
Hepatic Involvement ◽  
Serum And Urine

Abstract Objective To retrospectively identify the critical characteristics and prognostic factors of primary light-chain amyloidosis. Patients and Methods: Data were collected and compared from 91 patients who were diagnosed with primary light-chain amyloidosis at four hospitals between January 2010 and November 2018. We analyzed the clinical characteristics and performed an overall survival (OS) analysis. Results: Patients (median age, 60 years) were diagnosed with organ involvement of the kidney (91.2%), heart (56%), liver (14.3%), soft tissue (18.7%), or gastrointestinal tract (15.4%), and 68.1% of patients had more than two organs involved. Patients were most commonly treated with bortezomib-based regimens (56%), and only one patient had autologous stem cell transplantation (auto-ASCT). The median OS was 36.33 months and was affected by the ECOG score, renal involvement, cardiac involvement, hepatic involvement and negative immunofixation in the serum and urine after treatment. Multivariate analysis indicated that cardiac involvement and negative immunofixation in the serum and urine after treatment were independent prognostic factors for OS. Conclusion: Cardiac involvement and the hematologic response to treatment were independent prognostic factors for OS in primary light-chain amyloidosis patients. The type and number of organs involved is more important than the number of organs involved for the OS.

scholarly journals A Five-Gene Expression Signature Predicts Clinical Outcome of Ovarian Serous Cystadenocarcinoma

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Li-Wei Liu ◽  
Qiuhao Zhang ◽  
Wenna Guo ◽  
Kun Qian ◽  
Qiang Wang
Keyword(s):  
Gene Expression ◽  
Survival Time ◽  
Proportional Hazards ◽  
Gene Expression Signature ◽  
High Specificity ◽  
Cox Proportional Hazards ◽  
Assessment Model ◽  
Serous Cystadenocarcinoma ◽  
Specificity And Sensitivity ◽  
Risk Patients

Ovarian serous cystadenocarcinoma is a common malignant tumor of female genital organs. Treatment is generally less effective as patients are usually diagnosed in the late stage. Therefore, a well-designed prognostic marker provides valuable data for optimizing therapy. In this study, we analyzed 303 samples of ovarian serous cystadenocarcinoma and the corresponding RNA-seq data. We observed the correlation between gene expression and patients’ survival and eventually established a risk assessment model of five factors using Cox proportional hazards regression analysis. We found that the survival time in high-risk patients was significantly shorter than in low-risk patients in both training and testing sets after Kaplan-Meier analysis. The AUROC value was 0.67 when predicting the survival time in testing set, which indicates a relatively high specificity and sensitivity. The results suggest diagnostic and therapeutic applications of our five-gene model for ovarian serous cystadenocarcinoma.

Renal Heavy Chain and Heavy+Light Chain Amyloidosis: A Report of 17 Cases and Comparison with Renal Light Chain Amyloidosis

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3992-3992 ◽  
Author(s):  
Samih H. Nasr ◽  
Samar M. Said ◽  
Anthony M. Valeri ◽  
Sanjeev Sethi ◽  
Lynn D. Cornell ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Serum Creatinine ◽  
Light Chain ◽  
Cardiac Involvement ◽  
Plasma Cells ◽  
Kidney Biopsy ◽  
P Value ◽  
Fat Pad ◽  
Monoclonal Protein ◽  
Light Chain Amyloidosis

Abstract Abstract 3992 Little is known about the rare entities of heavy and light chain amyloidosis (AHL) and heavy chain amyloidosis (AH). In this study, we report the renal and hematologic characteristics, pathology, and outcome of 17 patients with renal AH/AHL including 5 with AH (4 IgG and 1 IgA) and 12 with AHL (7 IgGλ, 3 IgAκ, 1 IgAλ, and 1 IgMλ), and compare them with 202 patients with renal AL amyloidosis (AL) diagnosed during the same time period. All cases were diagnosed by kidney biopsy that showed Congo red-positive deposits. Amyloid typing was done by laser microdissection and mass spectrometry (LMD/MS) (12 patients) or by immunofluorescence (5 patients). All patients with renal AH/AHL were Caucasians, with a M:F ratio of 2.4 and a median age at biopsy of 63 years. Compared with patients with renal AL, those with renal AH/AHL had less frequent concurrent cardiac involvement, higher likelihood of having circulating complete monoclonal Ig, lower sensitivity of fat pad biopsy and bone marrow biopsy for detecting amyloid, higher incidence of hematuria, and better patient survival. The hematologic and renal responses to chemotherapy were comparable to renal AL. In 42% of patients, AH/AHL could not have been diagnosed without LMD/MS. In conclusion, renal AH/AHL is an uncommon but under-recognized form of amyloidosis, and its diagnosis is greatly enhanced by the use of LMD/MS for amyloid typing. The accurate histological diagnosis of renal AH/AHL and distinction from AL may have important clinical and prognostic implications. Table 1. Demographics and hematologic characteristics AH/AHL AL p value No. of patients 17 202 Gender: Male/female 12/5 (71%/29%) 126/76 (62%/38%) 0.61 Age, median (range) 63 (50–77) 62 (36–86) 0.73 Additional organ involvement 8 (47%) 126 (62%) 0.3 Cardiac involvement 3 (18%) 100 (50%) 0.01* % of plasma cells in bone marrow, median (IQR) 8 (5–15) 6 (5–10) 0.82     ≥30 plasma cells 4 (24%) 11/198 (6%) 0.02* Positive SPEP/SIF for paraprotein 15 (88%) 158/200 (79%) 0.53     Presence of whole monoclonal protein on SPEP 14 (82%) 108/200 (54%) 0.04* Positive UPEP/UIF for paraprotein 13/16 (81%) 158/189 (84%) 0.73     Presence of whole monoclonal protein on UPEP 10/16 (63%) 61/189 (32%) 0.03* Abnormal serum FLC ratio (<0.26 or >1.65) 9/12 (75%) 150/188 (80%) 0.71 Markedly abnormal FLC ratio (< 0.125 or > 8) 5/12 (42%) 100/188 (53%) 0.55 Positive bone marrow for amyloid 6/16 (38%) 135/183 (74%) 0.004* Positive fat pad biopsy for amyloid 2/14 (14%) 105/145 (72%) <0.001* IQR, interquartile range. Table 2. Renal characteristics at kidney biopsy AH/AHL AL p value No. of patients 17 202 24h urine protein in g, median (IQR) 5.1 (3.2–9.0) 6.0 (3.2–10.0) 0.9 Full nephrotic syndrome 9/16 (56%) 132/197 (67%) 0.42 Serum albumin in g/dl, median (IQR) 2.7 (2.2–3.3) 2.5 (1.9–2.9) 0.29 % albuminuria on UPEP, median (IQR) 68 (61–72) 70 (60–76) 0.47 Serum creatinine in mg/dl, median (IQR) 1.4 (1.1–2.1) 1.2 (0.9–1.8) 0.25 Serum creatinine >1.2 mg/dl 10/16 (63%) 92/201 (46%) 0.3 eGFR, median (IQR) 47 (27–67) 58 (36–75) 0.29 Decreased eGFR 10/16 (63%) 103/201 (51%) 0.44 Microscopic hematuria 9/16 (56%) 44/169 (26%) 0.02* IQR, interquartile range. Disclosures: No relevant conflicts of interest to declare.

Prognostic factors for survival in patients with stage (stg) IV malignant melanoma (MM)

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 9072-9072
Author(s):  
N. Seetharamu ◽  
H. Hamilton ◽  
T. Tu ◽  
P. Christos ◽  
I. Osman ◽  
...  
Keyword(s):  
Proportional Hazards ◽  
Univariate Analysis ◽  
Primary Melanoma ◽  
Stage Iv ◽  
Female Gender ◽  
Cox Proportional Hazards ◽  
Independent Effect ◽  
P Value ◽  
Related Factors ◽  
Ajcc Staging

9072 Background: Prognosis for survival in MM is not uniform with some pts being long-term survivors. Identifying this subset of pts may have implications on surveillance and treatment (tx). Unfortunately, prognostic data available for MM and the utility of AJCC staging in predicting survival is limited. We analyzed prospectively collected data from the NYUCI Interdisciplinary Melanoma Cooperative Group program (IMCG) to identify clinicopathological variables predictive of MM survival. Methods: We identified 185 pts enrolled in the IMCG with MM diagnosed and treated at NYUCI. Demographic, clinical, and tx-related factors were included in the analysis. Kaplan-Meier (KM) survival analysis was used to identify univariate predictors of post-stage IV survival and their independent effect was assessed in a multivariate Cox proportional hazards regression model. Results: Median age at diagnosis (dx) of metastatic MM was 64 years (22–92). Median overall survival: 13.8 months(m) (128 deaths and a median follow up of 18.6 m (4–141) for survivors). Factors identified on univariate analysis at p<0.20 were evaluated in the multivariate model ( table ). Co-morbidities, site and histology of primary melanoma, initial staging, prior loco-regional recurrences, and adjuvant tx of primary melanoma were not associated with MM survival. Univariate analysis also showed significant survival advantage (p value 0.0011) for patients with AJCC stages M1a and M1b (21.6 m and 17.2 m respectively) over those with AJCC stage M1c (9 m). Conclusions: This cohort study of MM identified female gender, nl serum LDH, nl albumin, and solitary organ involvement as independent survival predictors. Patients who received systemic therapy± local measures had survival benefit over those that had surgery and/or radiation alone suggesting a role for systemic treatment in MM. Patients with personal history of another malignancy (n=37) showed a trend towards improved survival. This novel observation needs to be validated and studied further. [Table: see text] No significant financial relationships to disclose.

Sign in / Sign up

Export Citation Format

Share Document