Abstract
According to the updated International Myeloma Working Group criteria, smoldering multiple myeloma (SMM) is an asymptomatic plasma cell disorder characterized by an M-component >3 g/dL, bone marrow plasma cell infiltration >10% and <60%, and absence of any myeloma-defining event. Active multiple myeloma is preceded by SMM, with a median time to progression of approximately 5 years. Cases of SMM range from the extremes of “monoclonal gammopathy of undetermined significance-like”, in which patients never progress during their lifetimes, to “early multiple myeloma”, in which transformation into symptomatic disease, based on genomic evolution, may be rapid and devastating. Such a “split personality” makes the prognosis and management of individual patients challenging, particularly with regard to the identification and possible early treatment of high-risk SMM. Outside of clinical trials, the conventional approach to SMM generally remains close observation until progression to active multiple myeloma. However, two prospective, randomized trials have recently demonstrated a significant clinical benefit in terms of time to progression, and of overall survival in one of the two studies, for some patients with higher-risk SMM treated with lenalidomide ± dexamethasone, raising the question of whether such an approach should be considered a new standard of care. In this paper, experts from the European Myeloma Network describe current biological and clinical knowledge on SMM, focusing on novel insights into its molecular pathogenesis, new prognostic scoring systems proposed to identify SMM patients at higher risk of early transformation, and updated results of completed or ongoing clinical trials. Finally, some practical recommendations for the real-life management of these patients, based on Delphi consensus methodology, are provided.
Originalsprog | Engelsk |
---|---|
Tidsskrift | Haematologica |
Vol/bind | 106 |
Udgave nummer | 11 |
Sider (fra-til) | 2799-2812 |
ISSN | 0390-6078 |
DOI | |
Status | Udgivet - 2021 |
Bibliografisk note
Publisher Copyright:©2021 Ferrata Storti Foundation
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2021 European myeloma network review and consensus statement on smoldering multiple myeloma : How to distinguish (and manage) Dr. Jekyll and Mr. Hyde. / Musto, Pellegrino; Engelhardt, Monika; Caers, Jo; Bolli, Niccolo; Kaiser, Martin; van de Donk, Niels; Terpos, Evangelos; Broijl, Annemiek; de Larrea, Carlos Fernández; Gay, Francesca; Goldschmidt, Hartmut; Hajek, Roman; Vangsted, Annette Juul; Zamagni, Elena; Zweegman, Sonja; Cavo, Michele; Dimopoulos, Meletios; Einsele, Hermann; Ludwig, Heinz; Barosi, Giovanni; Boccadoro, Mario; Mateos, Maria Victoria; Sonneveld, Pieter; Miguel, Jesus San.
I: Haematologica, Bind 106, Nr. 11, 2021, s. 2799-2812.Publikation: Bidrag til tidsskrift › Review › Forskning › peer review
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TY - JOUR
T1 - 2021 European myeloma network review and consensus statement on smoldering multiple myeloma
T2 - How to distinguish (and manage) Dr. Jekyll and Mr. Hyde
AU - Musto, Pellegrino
AU - Engelhardt, Monika
AU - Caers, Jo
AU - Bolli, Niccolo
AU - Kaiser, Martin
AU - van de Donk, Niels
AU - Terpos, Evangelos
AU - Broijl, Annemiek
AU - de Larrea, Carlos Fernández
AU - Gay, Francesca
AU - Goldschmidt, Hartmut
AU - Hajek, Roman
AU - Vangsted, Annette Juul
AU - Zamagni, Elena
AU - Zweegman, Sonja
AU - Cavo, Michele
AU - Dimopoulos, Meletios
AU - Einsele, Hermann
AU - Ludwig, Heinz
AU - Barosi, Giovanni
AU - Boccadoro, Mario
AU - Mateos, Maria Victoria
AU - Sonneveld, Pieter
AU - Miguel, Jesus San
N1 - Funding Information: PM has served as a member of advisory boards and/or received honoraria from Celgene, Janssen, Takeda, Bristol-Myers Squibb, Amgen, Novartis, Gilead, Jazz, Sanofi, Abbvie, and Glaxo-Smith-Kline. NB has received honoraria from Celgene, Takeda, Janssen, and Amgen; and served on an advisory board for Janssen. MK has received consultancy fees from AbbVie, Amgen, BMS/Celgene, GSK, Janssen, Karyopharm, Seattle Genetics, and Takeda; honoraria from BMS/Celgene, Janssen, and Takeda; research funding from Janssen (to his institution), BMS/Celgene (to his institution); and travel support from BMS/Celgene, Janssen, and Takeda. NVDD has received research support from Janssen Pharmaceuticals, Amgen, Celgene, Novartis, and BMS; and served on advisory boards for Janssen Pharmaceuticals, Amgen, Celgene, BMS, Takeda, Roche, Novartis, Bayer, and Servier. ET has received consultancy fees and honoraria from Amgen, BMS, Janssen, Celgene, Takeda, Genesis Pharma, GSK and Sanofi; and research support from Amgen, Janssen, Celgene, Genesis Pharma, GSK and Sanofi. FG has received honoraria from Amgen, Celgene, Janssen, Takeda, BMS, AbbVie, and GSK; and has served on advisory boards for Amgen, Celgene, Janssen, Takeda, BMS, AbbVie, GSK, Roche, Adaptive Biotechnologies, and Oncopeptides. HG has received grants and/or provision of investigational medicinal products from Amgen, BMS, Celgene, Chugai, Dietmar-Hopp-Foundation, Janssen, John Hopkins University, Sanofi; research support (institutions) from Amgen, BMS, Celgene, Chugai, Janssen, Incyte, Molecular Partners, Merck Sharp and Dohme (MSD), Sanofi, Mundipharma, Takeda, and Novartis; served on advisory boards for Adaptive Biotechnology, Amgen, BMS, Celgene, Janssen, Sanofi, and Takeda; and honoraria (for speakers bureaus) from Academy2 GmbH & Co. KG, Agentur Hogg Robinson Germany, Amgen, ArtTempi, Beupdated Helbig Consulting and Research AG Schweiz, BMS, Celgene, Chop GmbH, Chugai, Congress Culture Concept Dr. S. Stocker München, Connectmedia Warschau/Polen, Dr. Hubmann Tumorzentrum München, FomF GmbH, GlaxoSmithKline (GSK), GWT Forschung und Innovation Dresden, Institut für Versorgungsforschung in der Onkologie GbR, Janssen, Kompetenznetz Maligne Lymphome (KML) e.V., MedConcept GmbH, Medical Communication Gmbh, Münchner Leukämie Labor Prof. Haferlach, New Concept Oncology, Novartis, Omnia Med Deutschland, Onko Internetportal dkg-web GmbH, Sanofi, STIL Forschungs GmbH, and Veranstaltungskonzept Gesundheit Mechernich. RH has received research funding from Janssen, Amgen, Celgene, BMS, Novartis and Takeda; served on advisory boards for Janssen, Amgen, Celgene, AbbVie, BMS, Novartis, PharmaMar and Takeda; and has received honoraria from Janssen, Amgen, Celgene, BMS, Pharma Mar and Takeda. EZ has received honoraria from and served on advisory boards for Janssen, BMS, Takeda, Sanofi, Oncopeptide, GSK, and Amgen. SZ has received research funding from Takeda and Janssen; and served on advisory boards for Celgene, Takeda, Janssen, Sanofi and Oncopeptides. MC has received honoraria from Janssen, Celgene, Amgen, BMS, Takeda, AbbVie, Sanofi, and Adaptive Biotechnologies, and is a member of speakers’ bureaus for Janssen and Celgene. MD has received consultancy fees and honoraria from Janssen, Celgene, Takeda, Amgen and BMS. HL has received research funding from Amgen, Takeda; and served on speaker’s bureau/advisory boards for Amgen, Takeda, Sanofi, Celgene-BMS, Seattle Genetics, and Janssen. MB has received honoraria from Sanofi, Celgene, Amgen, Janssen, Novartis, BMS, and AbbVie; served on advisory boards for Janssen and GSK; and has received research funding from Sanofi, Celgene, Amgen, Janssen, Novartis, BMS, and Mundipharma. M-VM has served on advisory boards for or received honoraria from Janssen, BMS, Celgene, Takeda, Amgen, Sanofi, Oncopeptides, GSK, Adaptive, Pfizer, Regeneron, Roche and Sea-Gen. PS has received honoraria and research funding from Amgen, Celgene, Janssen, SkylineDx, and Takeda; JSM has received consultancy fees from Amgen, BMS, Celgene, Janssen, MSD, Novartis, GSK Takeda, Sanofi, and Roche. Publisher Copyright: ©2021 Ferrata Storti Foundation
PY - 2021
Y1 - 2021
N2 - According to the updated International Myeloma Working Group criteria, smoldering multiple myeloma (SMM) is an asymptomatic plasma cell disorder characterized by an M-component >3 g/dL, bone marrow plasma cell infiltration >10% and <60%, and absence of any myeloma-defining event. Active multiple myeloma is preceded by SMM, with a median time to progression of approximately 5 years. Cases of SMM range from the extremes of “monoclonal gammopathy of undetermined significance-like”, in which patients never progress during their lifetimes, to “early multiple myeloma”, in which transformation into symptomatic disease, based on genomic evolution, may be rapid and devastating. Such a “split personality” makes the prognosis and management of individual patients challenging, particularly with regard to the identification and possible early treatment of high-risk SMM. Outside of clinical trials, the conventional approach to SMM generally remains close observation until progression to active multiple myeloma. However, two prospective, randomized trials have recently demonstrated a significant clinical benefit in terms of time to progression, and of overall survival in one of the two studies, for some patients with higher-risk SMM treated with lenalidomide ± dexamethasone, raising the question of whether such an approach should be considered a new standard of care. In this paper, experts from the European Myeloma Network describe current biological and clinical knowledge on SMM, focusing on novel insights into its molecular pathogenesis, new prognostic scoring systems proposed to identify SMM patients at higher risk of early transformation, and updated results of completed or ongoing clinical trials. Finally, some practical recommendations for the real-life management of these patients, based on Delphi consensus methodology, are provided.
AB - According to the updated International Myeloma Working Group criteria, smoldering multiple myeloma (SMM) is an asymptomatic plasma cell disorder characterized by an M-component >3 g/dL, bone marrow plasma cell infiltration >10% and <60%, and absence of any myeloma-defining event. Active multiple myeloma is preceded by SMM, with a median time to progression of approximately 5 years. Cases of SMM range from the extremes of “monoclonal gammopathy of undetermined significance-like”, in which patients never progress during their lifetimes, to “early multiple myeloma”, in which transformation into symptomatic disease, based on genomic evolution, may be rapid and devastating. Such a “split personality” makes the prognosis and management of individual patients challenging, particularly with regard to the identification and possible early treatment of high-risk SMM. Outside of clinical trials, the conventional approach to SMM generally remains close observation until progression to active multiple myeloma. However, two prospective, randomized trials have recently demonstrated a significant clinical benefit in terms of time to progression, and of overall survival in one of the two studies, for some patients with higher-risk SMM treated with lenalidomide ± dexamethasone, raising the question of whether such an approach should be considered a new standard of care. In this paper, experts from the European Myeloma Network describe current biological and clinical knowledge on SMM, focusing on novel insights into its molecular pathogenesis, new prognostic scoring systems proposed to identify SMM patients at higher risk of early transformation, and updated results of completed or ongoing clinical trials. Finally, some practical recommendations for the real-life management of these patients, based on Delphi consensus methodology, are provided.
U2 - 10.3324/haematol.2021.278519
DO - 10.3324/haematol.2021.278519
M3 - Review
C2 - 34261295
AN - SCOPUS:85119072264
VL - 106
SP - 2799
EP - 2812
JO - Haematologica
JF - Haematologica
SN - 0390-6078
IS - 11
ER -