Monoclonal gammopathy of clinical significance: a novel concept with therapeutic implications
Monoclonal gammopathy of clinical significance: a novel concept with therapeutic implications
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DOI：
10.1182/blood-2018-04-839480
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发表时间：
2018-10-04
期刊：
影响因子：
20.3
通讯作者：
Merlini, Giampaolo
中科院分区：
文献类型：
--
作者：
Fermand, Jean-Paul;Bridoux, Frank;Merlini, Giampaolo
单克隆丙种球蛋白病是一种常见病，尤其是老年人。它可能表明有症状的多发性骨髓瘤或其他需要立即化疗的明显恶性淋巴疾病。更常见的是，它是由小型和/或静态分泌 B 细胞克隆引起的，完全无症状，仅需要定期监测，定义为意义不明的单克隆丙种球蛋白病 (MGUS)。有时，尽管克隆处于静止状态并且本身不需要任何治疗，但由于单克隆免疫球蛋白的毒性或其他机制，克隆与潜在的严重器官损伤有关。后一种情况越来越多地被观察到，但仍然认识不足，并且经常治疗不足，尽管它通常需要快速的特定干预来保护相关器官功能。为了提高对这些小 B 细胞克隆相关疾病的早期识别和管理，我们建议引入具有临床意义的单克隆丙种球蛋白病 (MGCS) 的概念。本报告确定了根据组织损伤机制分类的 MGCS 谱。它强调了这些疾病的多样性，这些疾病的诊断和治疗在临床实践中往往具有挑战性，需要采用多学科方法。还描述了管理原则，包括主要的诊断和治疗程序。重要的是，对基础 B 细胞克隆的有效控制通常会导致器官改善。目前，它主要依靠化疗和其他抗B细胞/浆细胞药物，其目标是快速产生最佳的血液学反应。
Monoclonal gammopathy is a common condition, particularly in the elderly. It can indicate symptomatic multiple myeloma or another overt malignant lymphoid disorder requiring immediate chemotherapy. More frequently, it results from a small and/or quiescent secreting B-cell clone, is completely asymptomatic, and requires regular monitoring only, defining a monoclonal gammopathy of unknown significance (MGUS). Sometimes, although quiescent and not requiring any treatment per se, the clone is associated with potentially severe organ damage due to the toxicity of the monoclonal immunoglobulin or to other mechanisms. The latter situation is increasingly observed but still poorly recognized and frequently undertreated, although it often requires rapid specific intervention to preserve involved organ function. To improve early recognition and management of these small B-cell clone-related disorders, we propose to introduce the concept of monoclonal gammopathy of clinical significance (MGCS). This report identifies the spectrum of MGCSs that are classified according to mechanisms of tissue injury. It highlights the diversity of these disorders for which diagnosis and treatment are often challenging in clinical practice and require a multidisciplinary approach. Principles of management, including main diagnostic and therapeutic procedures, are also described. Importantly, efficient control of the underlying B-cell clone usually results in organ improvement. Currently, it relies mainly on chemotherapy and other anti-B-cell/plasma cell agents, which should aim at rapidly producing the best hematological response.