scholarly journals A prognostic staging system for light-chain amyloidosis using hepatic and renal indicator data from 1,064 Chinese patients

2021 ◽  
Vol 0 (0) ◽  
pp. 0-0
Author(s):  
Wei Yan ◽  
Yanze Cao ◽  
Aijun Liao ◽  
Wei Yang ◽  
Jian Li ◽  
...  
2019 ◽  
Author(s):  
Donghua He ◽  
Fangshu Guan ◽  
Minli Hu ◽  
Gaofeng Zheng ◽  
Pan Hong ◽  
...  

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.


2012 ◽  
Vol 28 (3) ◽  
pp. 689-697 ◽  
Author(s):  
Ying Yao ◽  
Su-Xia Wang ◽  
You-Kang Zhang ◽  
Zhen Qu ◽  
Gang Liu ◽  
...  

Author(s):  
Donghua He ◽  
Fangshu Guan ◽  
Minli Hu ◽  
Gaofeng Zheng ◽  
Jingsong He ◽  
...  

AbstractTo retrospectively identify the critical characteristics and prognostic factors of light-chain amyloidosis. Patients and Methods: Data were collected and compared from 91 patients who were diagnosed with 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 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 influenced by the ECOG score, renal involvement, cardiac involvement, hepatic involvement, and persistence of positive immunofixation. Patients who received bortezomib-based treatment had a trend of favorable OS compared to those who received non-bortezomib-based treatments, but the difference was not statistically significant. Although the overall number of organs involved was not related to OS, the number of organs involved in the heart, liver and kidney was related. Multivariate analysis indicated that cardiac involvement and negative hematologic response with persistence of positive immunofixation were independent prognostic factors for OS. Conclusion: Cardiac involvement and the hematologic response to treatment were independent prognostic factors for OS in light-chain amyloidosis patients.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 599-599
Author(s):  
Morie A Gertz ◽  
Martha Q Lacy ◽  
Angela Dispenzieri ◽  
Shaji K. Kumar ◽  
David Dingli ◽  
...  

Abstract Abstract 599 Purpose: This study was undertaken to develop selection guidelines for transplant centers to determine eligibility for stem cell transplantation in patients with light chain amyloidosis. Patients and Methods: Patients with biopsy-confirmed immunoglobulin light chain amyloidosis who underwent stem cell transplantation between March 8, 1996, and December 31, 2011, were reviewed in 2 cohorts: those who underwent transplantation between March 8, 1996, and June 30, 2009, and those who underwent transplantation between July 1, 2009, and December 31, 2011 (table 1). A second comparison was undertaken among patients who died before posttransplant day 100 to determine features predictive of early death (table 2). Results: A total of 499 patients were identified, 410 in the earlier group and 89 in the later group. After July 1, 2009, significantly fewer transplant recipients had Mayo stage III cardiac involvement. Mortality before posttransplant day 100 was 10.5% (43/410) in the earlier group and 1.1% (1/89) in the later group. In the earlier group, one-quarter of transplant recipients with N-terminal pro-brain natriuretic peptide (NT-proBNP) levels higher than 5,000 pg/mL died by 10.3 months (fig 1, left). When the serum troponin T level was > 0.06 ng/mL, 25% died at 3.7 months (fig 2, right). Conclusion: The Mayo staging system is highly predictive for overall survival but not useful for selecting transplant recipients. Patients with serum troponin T levels higher than 0.06 ng/mL or NT-proBNP levels >5,000 pg/mL (not on dialysis) should not be considered acceptable candidates for stem cell transplantation because of unacceptable early mortality. Disclosures: No relevant conflicts of interest to declare.


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