Diagnosis and Staging of Multiple Myeloma and Related Disorders
Diagnosis and Staging of Multiple Myeloma and Related Disorders
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DOI：
10.1007/978-3-319-25586-6_2
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发表时间：
2018-01-01
期刊：
影响因子：
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通讯作者：
San Miguel, Jesus F.
中科院分区：
文献类型：
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作者：
Rajkumar, S. Vincent;Fonseca, Rafael;San Miguel, Jesus F.
多发性骨髓瘤是一种浆细胞肿瘤，主要发生在老年人中，具有良性阶段，即所谓的 MGUS 和冒烟性骨髓瘤。浆细胞肿瘤各个阶段的精确定义对于决定何时开始治疗至关重要。传统上，当患者有明显的终末器官损伤证据时开始治疗，最好记住缩写词 CRAB。临床特征包括骨质破坏、高钙血症、肾功能不全或贫血的证据。最近，国际骨髓瘤工作组将具有高进展风险（2 年 80%）的高危冒烟性骨髓瘤患者重新分类为活动性骨髓瘤，需要治疗。这三个扩展标准包括血清游离轻链比率的极端偏差（FLC比率≥100，最小涉及的FLC水平至少为100mg/L）、MRI上有两个或更多病变，以及严重的浆细胞增多症（≥60％）。这些标准使我们能够在终末器官损伤之前开始治疗，有望防止患者出现长期并发症。除了标准临床标准外，医生还应该测试该疾病的遗传亚型。了解各种遗传亚型可以更好地对患者进行预后分类，并且最近可以为该疾病的亚型开发特定的治疗方法。
Multiple myeloma is a plasma cell neoplasm that predominantly occurs in the elderly, which has preceding benign stages, the so-called MGUS and smoldering myeloma. The precise definition of the various stages of the plasma cell neoplasms is critical to decide when therapy initiation is indicated. Traditionally, therapy was started when patients had overt evidence of end-organ damage, best remembered by the acronym CRAB. Clinical features included evidence of bone destruction, hypercalcemia, renal insufficiency, or anemia. More recently the International Myeloma Working Group has reclassified high-risk smoldering myeloma patients, at high risk of progression (80% at 2 years), as active myeloma and in need of therapy. The three expanded criteria include extreme deviation in the serum free light chain ratio (FLC ratio ≥100, with a minimal involved FLC level of at least 100 mg/L), two or more lesions on MRI, and also an extreme plasmacytosis (≥60%). These criteria allow us to initiate therapy before end-organ damage hopefully preventing long-lasting complications for patients. In addition to the standard clinical criteria, physicians should test for the genetic subtypes of the disease. Understanding the various genetic subtypes has allowed for a better prognostic categorization of patients and most recently has allowed for the development of specific therapeutic approaches for subsets of the disease.