1. Expert recommendation from the Swiss Amyloidosis Network (SAN) for systemic AL-amyloidosis
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Robert Manka, Alessio Mylonas, Andreas J. Flammer, Rene Nkoulou, Aju P. Pazhenkottil, Alexandar Tzankov, Natallia Laptseva, Andrea Cavalli, Violeta Mihaylova, Bernhard Gerber, Pierre Monney, Thomas Fehr, Friederike Vetter, Rahel Schwotzer, Stefan W Dobner, Manuela Averaimo, Raphael Heimgartner, Ariana Gaspert, Adrian Schmidt, Giorgio Treglia, Paolo Arosio, Stefan Dirnhofer, Luca Mazzucchelli, Thomas M. Suter, Chiara Kessler, Harald Seeger, Simon F. Stämpfli, Thomas Pabst, Vera Ulrike Bacher, Peter K. Bode, Axel Rüfer, Marie Théaudin, Guido Stirnimann, Adalgisa Concoluci, Annemarie Hübers, Markus Zweier, Sabine Gerull, Raphael Knöpfel, Nadia Djerbi, Martin Meyer, Maura Garofalo, Hans H. Jung, and Otmar Pfister
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medicine.medical_specialty ,MEDLINE ,expert recommendation ,610 Medicine & health ,treatment outcomes ,Disease ,diagnostic work-up and treatment ,03 medical and health sciences ,0302 clinical medicine ,cardiac biomarkers ,Plasma Cell Myeloma ,staging system ,medicine ,AL amyloidosis ,Humans ,Immunoglobulin Light-chain Amyloidosis ,swiss amyloidosis network ,undetermined significance ,al amyloidosis ,stem-cell transplantation ,factor-x deficiency ,Intensive care medicine ,Multiple myeloma ,high-dose melphalan ,light-chain amyloidosis ,Swiss Amyloidosis Network ,Expert recommendation ,Diagnostic work-up and treatment ,biology ,business.industry ,monoclonal gammopathy ,Amyloidosis ,General Medicine ,medicine.disease ,Transthyretin ,multiple-myeloma ,030220 oncology & carcinogenesis ,biology.protein ,Multiple Myeloma ,business ,Switzerland ,030215 immunology ,Rare disease - Abstract
Systemic amyloidosis is a heterogeneous group of diseases associated with protein misfolding into insoluble beta-sheet rich structures that deposit extracellularly in different organs, eventually compromising their function. There are more than 30 different proteins, known to be amyloidogenic with “light chain” (AL)-amyloidosis being the most common type, followed by transthyretin (ATTR)-, and amyloid protein A (AA)-amyloidosis. Systemic amyloidosis is a rare disease with an incidence of around 10 patients in 1 million inhabitants. Recently several new therapeutic options have been developed for subgroups of amyloidosis patients, and the introduction of novel therapies for plasma cell myeloma has led to an increase in the therapeutic armamentarium for plasma cell disorders, including AL amyloidosis. Among them, proteasome inhibitors, immunomodulatory agents (-imids), and monoclonal antibodies have been successfully introduced into clinical practice. Still, high-quality data from randomised controlled trials regarding the benefit of these cost-intensive drugs in AL amyloidosis are widely lacking, and due to the rarity of the disease many physicians will not gain routine experience in the management of these frail patients. The diagnosis of AL amyloidosis relies on a close collaboration between clinicians, pathologists, imaging experts, and sometimes geneticists. Diagnosis and treatment options in this complex disorder should be discussed in dedicated multidisciplinary boards. In January 2020, the first meeting of the Swiss Amyloidosis Network took place in Zurich, Switzerland. One aim of this meeting was to establish a consensus guideline regarding the diagnostic work-up and the treatment recommendations for systemic amyloidosis tailored to the Swiss health care system. Forty-five participants from different fields in medicine discussed many aspects of amyloidosis. These are the Swiss Amyloidosis Network recommendations which focus on diagnostic work-up and treatment of AL-amyloidosis., Swiss Medical Weekly, 150, ISSN:1424-7860, ISSN:1424-3997
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- 2020
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