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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.
摘要：
根据最新的国际骨髓瘤工作组标准，闷烧的多发性骨髓瘤（SMM）是一种无症状的浆细胞疾病，其特征是M成分>3g/dL，骨髓浆细胞浸润>10%和<60%，并且没有任何骨髓瘤定义事件。活动性多发性骨髓瘤先有SMM，中位进展时间约为5年。SMM的病例范围从“不确定的显著性样单克隆丙种球蛋白病”的极端开始，患者一生中从未进步，到“早期多发性骨髓瘤”，转变为有症状的疾病，基于基因组进化，可能是快速和毁灭性的。这种“分裂的人格”使个体患者的预后和管理具有挑战性，特别是关于高危SMM的识别和可能的早期治疗。在临床试验之外,在进展为活动性多发性骨髓瘤之前,SMM的常规方法通常仍需密切观察.然而,两个潜在的，随机试验最近证明在进展时间方面具有显著的临床益处，以及两项研究之一的总体生存率，对于一些接受来那度胺±地塞米松治疗的高危SMM患者，提出了这样一种方法是否应该被视为一种新的护理标准的问题。在本文中,来自欧洲骨髓瘤网络的专家描述了SMM的当前生物学和临床知识，专注于对其分子发病机理的新颖见解，提出了新的预后评分系统，用于识别早期转化风险较高的SMM患者，以及已完成或正在进行的临床试验的最新结果。最后,为这些患者的实际生活管理提供一些实用建议，基于德尔菲共识方法，提供。
