Expert recommendation from the Swiss Amyloidosis Network (SAN) for systemic AL-amyloidosis

Schwotzer, Rahel; Flammer, Andreas J.; Gerull, Sabine; Pabst, Thomas; Arosio, Paolo; Averaimo, Manuela; Bacher, Vera Ulrike; Bode, P.; Cavalli, Andrea; Concoluci, Adalgisa; Dirnhofer, Stefan; Djerbi, Nadia; Dobner, Stephan; Fehr, Thomas; Garofalo, Maura; Gaspert, Ariana; Heimgartner, Raphael; Hübers, Annemarie; Jung, Hans H.; Kessler, Chiara; Knöpfel, Raphael; Laptseva, Natallia; Manka, Robert; Mazzucchelli, Luca; Meyer, Martin; Mihaylova, Violeta; Monney, Pierre; Mylonas, Alessio; Nkoulou, René; Pazhenkottil, Aju P.; Pfister, Otmar; Rüfer, Axel; Schmidt, Adrian; Seeger, Harald; Stämpfli, Simon F.; Stirnimann, Guido; Suter, Thomas M.; Théaudin, Marie; Treglia, Giorgio; Tzankov, Alexandar; Vetter, Friederike; Zweier, Christiane; Gerber, Bernhard

doi:10.4414/smw.2020.20364

Cited by 18 publications

(16 citation statements)

References 88 publications

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“…However, based on numerous Phase II trials, a general consensus has evolved, with patients deemed fit taken to autologous transplant, often after induction therapy. One example of such a consensus approach is an algorithm for treatment published by the Swiss Amyloidosis Network [44]. This group currently recommends induction therapy using the combination of cyclophosphamide, bortezomib, and dexamethasone ("CyBorD") followed by high dose melphalan with autologous hematopoietic rescue, for transplant-eligible patients.…”

Section: Al Amyloidosismentioning

confidence: 99%

An Historical Overview of the Amyloidoses

Harrison¹,

Cohen²,

Ioffe³

et al. 2022

Amyloidosis - History and Perspectives

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The amyloidoses are a heterogenous group of clinical disorders that share the common finding of the abnormal deposition of insoluble proteins into various organs, with the result that these proteinaceous deposits disrupt cellular function and impair the integrity of the organs involved. Most typically, the abnormal protein deposition is the consequence of abnormal three dimensional folding of the culprit protein. The abnormal folding of the protein, in turn, may be due to a germ line mutation, may be due to an acquired mutation, or may be due to a polymorphism or characteristic of a normal protein that leads to abnormal folding, precipitation, and deposition of the protein, particularly when that protein is expressed at unusually high levels for a prolonged period of time. The clinical manifestations of an amyloid disorder are the consequences of the array of organs involved, the extent of amyloid deposition, and co-morbid conditions present in the individual patient. The array of organs involved, and the extent of organ involvement, in turn, depend in large part on the specific protein that is responsible for the amyloid deposition, and the process driving that protein’s production. In this chapter, a chronological overview is intended to summarize the critical insights into the patho-biology of amyloid accumulation of various types. These insights have allowed an improved understanding over time of the of the major subgroups and disease entities of the amyloidoses, leading to some degree of improvement in diagnosis and treatment outcomes. Unfortunately, as of this writing, treatment outcomes still remain poor for a large fraction of patients, and there is need for improvement in all aspects of the evaluation and management of these diseases.

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Section: Al Amyloidosismentioning

confidence: 99%

An Historical Overview of the Amyloidoses

Harrison¹,

Cohen²,

Ioffe³

et al. 2022

Amyloidosis - History and Perspectives

View full text Add to dashboard Cite

show abstract

“…91 diagnosis, Table 2 summarizes the main diagnostic algorithms published in the last few years. 1,7,51,[92][93][94][95][96][97] Overall, they emphasize the importance of clinical red flags, together with laboratory results, ECG, echocardiography and in most cases CMR for the initial screening.…”

Section: Future Directionsmentioning

confidence: 99%

“…Table 1 summarizes the main multimodality imaging findings that can help in the differentiation between AL and ATTR CA. Regarding diagnosis, Table 2 summarizes the main diagnostic algorithms published in the last few years 1,7,51,92–97 . Overall, they emphasize the importance of clinical red flags, together with laboratory results, ECG, echocardiography and in most cases CMR for the initial screening.…”

Section: Future Directionsmentioning

confidence: 99%

Multimodality imaging in cardiac amyloidosis: State‐of‐the‐art review

Timóteo

Rosa

Bras

et al. 2022

J of Clinical Ultrasound

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Amyloidosis is a systemic disease, characterized by deposition of amyloid fibrils in various organs, including the heart. For the diagnosis of cardiac amyloidosis (CA) it is required a high level of clinical suspicion and in the presence of clinical, laboratorial, and electrocardiographic red flags, a comprehensive multimodality imaging evaluation is warranted, including echocardiography, magnetic resonance, scintigraphy, and computed tomography, that will confirm diagnosis and define the CA subtype, which is of the utmost importance to plan a treatment strategy. We will review the use of multimodality imaging in the evaluation of CA, including the latest applications, and a practical flow‐chart will sum‐up this evidence.

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“…The Swiss Amyloidosis Network (SAN) recommends biannual screening of patients with MGUS and an abnormal FLC ratio, and with smoldering myeloma. 6 Even before overt heart failure and nephrotic syndrome occur, the measurement of N-terminal B-type natriuretic peptide (NT-proBNP) levels can detect cardiac amyloidosis, and albuminuria can be indicative of renal amyloidosis. These biomarkers can thus help to establish a diagnosis of AL amyloidosis prior to irreversible organ damage.…”

Section: F Ro M Pat H O P H Ys I O Lo Gy To B I O M a R K E R Smentioning

confidence: 99%

Systemic AL Amyloidosis and Precision Medicine

Schwotzer

2021

healthbook TIMES Onco Hema

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In amyloid light-chain (AL) amyloidosis, a small plasma-cell clone, or less frequently a mature B-cell clone, produces toxic monoclonal light chains that can be deposited in the tissues in the form of amyloid fibrils, which can affect the function of potentially multiple organs, including the heart and kidney. The mechanisms behind this organ function impairment are different from plasma-cell myeloma, as are the diagnostic work-up and the treatment strategies. Early pre-symptomatic diagnosis of AL amyloidosis is possible, and it is indeed crucial for effective therapy and the preservation of patients' quality of life. This is however challenging in the light of a lack of specific clinical symptoms or signs. In the case of suspicious clinical signs, along with the presence of monoclonal gammopathy and elevated validated biomarkers of organ involvement, further diagnostic workup should be initiated to confirm or exclude AL amyloidosis. Tissue typing of amyloid is also mandatory for the diagnosis of AL amyloidosis. Risk-stratification is essential to guide the therapeutic approach, which has to be individualized and take patient preferences into account. The aim of the therapy is rapid suppression of the production of light chains, to restore organ function, especially cardiac function. The severity of heart involvement and the burden of the pathological light chains are the main prognostic determinants in AL amyloidosis. This review discusses the diagnostic algorithms in suspected amyloidosis, the need for typing of the amyloid for prognostic and therapeutic reasons, and the recent developments in the risk-adapted treatment of AL amyloidosis. We further focus on anti-plasma-cell drugs and autologous stem-cell transplantation, as the tools for hematologic and organ response assessment.

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Expert recommendation from the Swiss Amyloidosis Network (SAN) for systemic AL-amyloidosis

Cited by 18 publications

References 88 publications

An Historical Overview of the Amyloidoses

An Historical Overview of the Amyloidoses

Multimodality imaging in cardiac amyloidosis: State‐of‐the‐art review

Systemic AL Amyloidosis and Precision Medicine

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