Clinical Characteristics and Treatment Outcome of Chinese Patients With Systemic Amyloid Light-Chain Amyloidosis: A Retrospective Single-Center Analysis

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.

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Clinical characteristics and treatment outcome of type I cryoglobulinemia in Chinese patients: a single-center study of 45 patients

Annals of Hematology ◽

10.1007/s00277-020-04123-1 ◽

2020 ◽

Vol 99 (8) ◽

pp. 1735-1740 ◽

Cited By ~ 1

Author(s):

Le-le Zhang ◽

Xin-xin Cao ◽

Kai-ni Shen ◽

Hong-xiao Han ◽

Cong-Li Zhang ◽

...

Keyword(s):

Treatment Outcome ◽

Clinical Characteristics ◽

Chinese Patients ◽

Type I ◽

Single Center ◽

Single Center Study ◽

Center Study

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Cyclophosphamide + Thalidomide + Dexamethasone Versus Melphalan + Dexamethasone for the Treatment of Amyloid Light-chain Amyloidosis With Kidney Involvement: A Retrospective Study in Chinese Patients

Clinical Therapeutics ◽

10.1016/j.clinthera.2018.12.003 ◽

2019 ◽

Vol 41 (6) ◽

pp. 1186-1198 ◽

Cited By ~ 2

Author(s):

Baojian Liu ◽

Yan Wang ◽

Ming Bai ◽

Di Wang ◽

Jin Zhao ◽

...

Keyword(s):

Retrospective Study ◽

Light Chain ◽

Chinese Patients ◽

Light Chain Amyloidosis ◽

Kidney Involvement ◽

Amyloid Light Chain

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A clinicopathological analysis in a large cohort of Chinese patients with renal amyloid light-chain amyloidosis

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfs501 ◽

2012 ◽

Vol 28 (3) ◽

pp. 689-697 ◽

Cited By ~ 8

Author(s):

Ying Yao ◽

Su-Xia Wang ◽

You-Kang Zhang ◽

Zhen Qu ◽

Gang Liu ◽

...

Keyword(s):

Light Chain ◽

Large Cohort ◽

Chinese Patients ◽

Light Chain Amyloidosis ◽

Clinicopathological Analysis ◽

Amyloid Light Chain

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Bortezomib-Containing Regimens for the Treatment of Newly Diagnosed and Relapsed Amyloid Light Chain Amyloidosis: A Single-Center Experience

Clinical Lymphoma Myeloma & Leukemia ◽

10.1016/j.clml.2016.03.005 ◽

2016 ◽

Vol 16 (6) ◽

pp. e79-e84 ◽

Cited By ~ 7

Author(s):

Victor H. Jimenez-Zepeda ◽

Peter Duggan ◽

Paola Neri ◽

Nizar J. Bahlis

Keyword(s):

Light Chain ◽

Newly Diagnosed ◽

Single Center ◽

Light Chain Amyloidosis ◽

Single Center Experience ◽

Amyloid Light Chain

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

Indian Journal of Hematology and Blood Transfusion ◽

10.1007/s12288-021-01469-y ◽

2021 ◽

Author(s):

Donghua He ◽

Fangshu Guan ◽

Minli Hu ◽

Gaofeng Zheng ◽

Jingsong He ◽

...

Keyword(s):

Prognostic Factors ◽

Light Chain ◽

Clinical Characteristics ◽

Cardiac Involvement ◽

Renal Involvement ◽

Chinese Patients ◽

Response To Treatment ◽

Light Chain Amyloidosis ◽

Hematologic Response ◽

Hepatic Involvement

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.

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Effects of Amyloid Light-chain Amyloidosis on Clinical Characteristics and Prognosis in Multiple Myeloma: A Single-center Retrospective Study

10.21203/rs.3.rs-49285/v1 ◽

2020 ◽

Author(s):

Junhui Xu ◽

Mangju Wang ◽

Ye Shen ◽

Miao Yan ◽

Weiwei Xie ◽

...

Keyword(s):

Multiple Myeloma ◽

Bone Marrow ◽

Light Chain ◽

Clinical Characteristics ◽

Al Amyloidosis ◽

Term Survival ◽

Bone Marrow Biopsies ◽

Poor Prognostic Factor ◽

Light Chain Amyloidosis ◽

Amyloid Light Chain

Abstract Background Amyloid light-chain amyloidosis (AL amyloidosis) is commonly associated with multiple myeloma. However, the clinical characteristics and prognosis of multiple myeloma with AL amyloidosis are not particularly clear. Methods Patients with multiple myeloma in the Peking University First Hospital registry from 2010 to 2018 were studied. The clinical and laboratory information were collected from first presentation to death or until the last available clinical follow-up. The patients’ survival and outcomes were analyzed, and the relationships between the clinical parameters and survival were also assessed. Results Compared with patients without AL amyloidosis, patients with AL amyloidosis had higher incidence of BNP ≧ 1000 pg/ml (P = 0.001), ALP > 187.5 IU/L (P = 0.002) and ALB < 35 g/L (P = 0.001), but lower incidence of HB < 85 g/L (P = 0.031), hypercalcemia > 2.65 mmol/L (P = 0.008), bone destruction more than three (P < 0.001), bone marrow plasma cell ratio ≧ 30% (P < 0.001) and worse D-S stage (P < 0.001). Multiple myeloma was more often complicated by λ-type AL amyloidosis than κ-type AL amyloidosis. However, further comparison found that multiple myeloma with κ-type AL amyloidosis had higher incidence of ALP > 187.5 IU/L (P = 0.001) and renal insufficiency (P = 0.001) along with worse D-S stage (P = 0.003) than multiple myeloma with λ-type AL amyloidosis. Renal biopsies of many patients suggested AL amyloidosis, but their bone marrow biopsies or subcutaneous abdominal fat pad aspirates results were negative. The existence of AL amyloidosis especially the heart involvement was related to shorter long-term survival of multiple myeloma according to univariate analysis. Cox regression model for overall survival detected the presence of AL amyloidosis in multiple myeloma having independent prognostic significance (RR = 4.52, P = 0.049). Conclusions Patients with multiple myeloma accompanied by AL amyloidosis have milder target organ damage and lower tumor burden. The incidence of AL amyloidosis in κ-type multiple myeloma is lower but more severe than λ-type multiple myeloma. Renal biopsy can help to identify patients with AL amyloidosis. AL amyloidosis is an independent poor prognostic factor for multiple myeloma is mainly because involvements of important organs especially the heart.

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