Clinical features and treatment outcome of primary systemic light‐chain amyloidosis in Korea: Results of multicenter analysis

Jun, Hyun Jung; Kım, Kihyun; Kim, Seok Jin; Mun, Yeung Chul; Bang, Soo‐Mee; Won, Jong-Ho; Kim, Chul Soo; Lee, Jae Hoon

doi:10.1002/ajh.23333

Cited by 15 publications

(15 citation statements)

References 22 publications

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“…Indeed, the clinical features and treatment outcomes were mainly correlated with the type and extent of organ involvement rather than by the number of plasma cells in patients with AL amyloidosis [24, 25]. By comparison, a previous study with longitudinal whole genome sequencing of a high-risk myeloma patients demonstrated tumor heterogeneity at diagnosis and identified potential mutations contributing to myeloma development as well as transformation from myeloma to overt extramedullary disease [26].…”

Section: Discussionmentioning

confidence: 99%

Recurrent mutations of MAPK pathway genes in multiple myeloma but not in amyloid light-chain amyloidosis

et al. 2016

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Clinically applicable platforms revealing actionable genomic alterations may improve the treatment efficacy of myeloma patients. In this pilot study, we used a high depth targeted sequencing panel containing 83 anti-cancer drug target genes to sequence genomic DNAs extracted from bone marrow aspirates of 23 patients with myeloma and 12 patients with amyloid light-chain (AL) amyloidosis. Mutation analysis revealed NRAS as the most commonly mutated gene (30%, 7/23) in myeloma patients followed by KRAS (26%, 6/23) and BRAF (22%, 5/23). However, no significant mutations were found in the 12 patients with AL amyloidosis. Notably, 6 of the 23 myeloma patients showed multi-site and/or multi-gene mutations in NRAS, KRAS, or BRAF, indicating compound aberrations in the Mitogen activated protein kinase (MAPK) pathway. Gene panel sequencing also revealed cytogenetic abnormalities associated with prognosis in myeloma patients. In conclusion, our pilot study suggests that targeted gene sequencing may have an important prognostic value for myeloma patients for the identification of actionable genomic alterations and cytogenetic aberrations.

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Section: Discussionmentioning

confidence: 99%

Recurrent mutations of MAPK pathway genes in multiple myeloma but not in amyloid light-chain amyloidosis

et al. 2016

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“…An association between the number of involved organs and mortality has been shown in patients with cardiac amyloidosis (11) and in overall AL amyloidosis patients (14). In the present study, the number of organs with amyloid involvement was selected as an independent predictor of mortality in patients with AL amyloidosis (Table 3).…”

Section: Discussionmentioning

confidence: 80%

“…Left and right ventricular (RV) functions assessed by conventional and advanced echocardiography [e.g., tissue Doppler echocardiography (TDE) and speckle tracking echocardiography] and cardiac biomarkers, such as cardiac troponin and brain natriuretic peptide (BNP), have been shown to be strong predictors of mortality in patients with AL amyloidosis (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15). However, the majority of previous studies have been conducted in Western countries (3)(4)(5)(6)(7)(8)(9)(10)(11), and the predictors of mortality in Asian patients with AL amyloidosis have not yet been characterized (12)(13)(14)(15).…”

Section: Introductionmentioning

confidence: 99%

“…Earlier studies (16,17) have shown that the response to treatment and the outcomes differ among heart failure patients with different ethnic backgrounds. Since the number of reports regarding the clinical characteristics of AL amyloidosis in the Asian population is small (12)(13)(14)(15), it is unclear whether there is an influence of ethnicity on the incidence of heart failure, its outcomes or the determinants of mortality in patients with AL amyloidosis. In the present study, we aimed to determine the predictors of mortality in Asian patients with biopsyconfirmed AL amyloidosis and to compare them with those reported in other ethnic populations.…”

Section: Introductionmentioning

confidence: 99%

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Pericardial Effusion and Multiple Organ Involvement Are Independent Predictors of Mortality in Patients with Systemic Light Chain Amyloidosis

et al. 2015

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Objective Left ventricular (LV) functions assessed by echocardiography and cardiac biomarkers are strong predictors of mortality in patients with systemic light chain (AL) amyloidosis. However, most previous studies have been conducted in Western countries, and the predictors of mortality in Asian patients with AL amyloidosis have not been characterized. To address this issue, we aimed to determine the predictors of mortality in Asian patients with biopsy-confirmed AL amyloidosis. Methods We retrospectively enrolled 31 patients (59±11 years, 55% men) in whom AL amyloidosis was confirmed by biopsies from cardiac or non-cardiac tissues. Of these patients, 15 (48%) met the international echocardiographic criteria for cardiac amyloidosis (mean LV wall thickness >12 mm without other causes of LV hypertrophy). Results During a mean follow-up period of 21±20 months, 15 patients died. Non-survivors had a higher number of involved organs, lower e', and higher rates of E/e' >15, pericardial effusion (PE), low voltage on an electrocardiogram and a New York Heart Association (NYHA) functional class !III, compared with survivors. In multivariate analysis, a NYHA functional class !III (p=0.024) and cardiac involvement (p=0.032) were independent predictors of PE in patients with AL amyloidosis. Multivariate Cox proportional hazard analysis indicated that PE (hazard ratio: 21.9, p=0.025) and the number of involved organs (hazard ratio: 2.8, p=0.015), but not LV diastolic parameters of tissue Doppler echocardiography, independently predict mortality in patients with AL amyloidosis. Conclusion PE and multiple organ involvement, compared with e' and E/e', are stronger predictors of mortality in patients with AL amyloidosis. The advanced disease stage of AL amyloidosis might underlie the strong association between PE and a poor outcome.

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“…The overall prognosis of pAL is poor, mainly because amyloid deposits in important organs such as the heart, liver and kidneys, and ultimately lead to organ failure and death. (4). In the last 15 years, substantial progress has been made in understanding the biology of the amyloid plasma cell clones and the mechanisms of organ damage; yet there are no specific treatments can be used to treat amyloidosis.…”

Section: Introductionmentioning

confidence: 99%

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

Guan

et al. 2019

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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 features and treatment outcome of primary systemic light‐chain amyloidosis in Korea: Results of multicenter analysis

Cited by 15 publications

References 22 publications

Recurrent mutations of MAPK pathway genes in multiple myeloma but not in amyloid light-chain amyloidosis

Recurrent mutations of MAPK pathway genes in multiple myeloma but not in amyloid light-chain amyloidosis

Pericardial Effusion and Multiple Organ Involvement Are Independent Predictors of Mortality in Patients with Systemic Light Chain Amyloidosis

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

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