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Extramedullary multiple myeloma.
Leukemia ( IF 12.8 ) Pub Date : 2019-11-27 , DOI: 10.1038/s41375-019-0660-0
Manisha Bhutani 1 , David M Foureau 2 , Shebli Atrash 1 , Peter M Voorhees 1 , Saad Z Usmani 1
Affiliation  

Extramedullary multiple myeloma (EMM) is an aggressive subentity of multiple myeloma, characterized by the ability of a subclone to thrive and grow independent of the bone marrow microenvironment, resulting in a high-risk state associated with increased proliferation, evasion of apoptosis and treatment resistance. Despite improvement in survival for most patients with multiple myeloma over recent decades, outcomes are generally poor when EMM develops. Understanding the molecular underpinnings leading to homing of plasma cells in ecosystems outside the bone marrow will be crucial for therapeutically manipulating the microenvironment and targeting key signaling pathways. Herein, we discuss the evolutionary biology of EMM, underscore the importance of a uniform definition, discuss prognostic significance, and provide current and emerging treatment strategies for managing this rare subentity of multiple myeloma.

中文翻译:

髓外多发性骨髓瘤。

髓外多发性骨髓瘤 (EMM) 是多发性骨髓瘤的一种侵袭性亚实体,其特征在于亚克隆能够独立于骨髓微环境而茁壮成长,导致与增殖增加、逃避细胞凋亡和治疗抵抗相关的高风险状态. 尽管近几十年来大多数多发性骨髓瘤患者的生存率有所提高,但当 EMM 发展时,结果通常很差。了解导致浆细胞在骨髓外生态系统中归巢的分子基础对于治疗性操纵微环境和靶向关键信号通路至关重要。在这里,我们讨论 EMM 的进化生物学,强调统一定义的重要性,讨论预后意义,
更新日期:2019-11-28
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