Dana-Farber Cancer Institute and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Jagiellonian University Medical College, Krakow, Poland.
Am J Hematol. 2017 Aug;92(8):746-751. doi: 10.1002/ajh.24753. Epub 2017 May 26.
IgM myeloma is a rare hematologic malignancy for which the clinicopathological features and patient outcomes have not been extensively studied. We carried out a multicenter retrospective study in patients with diagnosis of IgM myeloma defined by >10% marrow involvement by monoclonal plasma cells, presence of an IgM monoclonal paraproteinemia of any size, and anemia, renal dysfunction, hypercalcemia, lytic lesions and/or t(11;14) identified by FISH. A total of 134 patients from 20 centers were included in this analysis. The median age at diagnosis was 65.5 years with a male predominance (68%). Anemia, renal dysfunction, elevated calcium and skeletal lytic lesions were found in 37, 43, 19, and 70%, respectively. The median serum IgM level was 2,895 mg dL with 19% of patients presenting with levels >6,000 mg dL . International Staging System (ISS) stages 1, 2, and 3 were seen in 40 (33%), 54 (44%), and 29 (24%) of patients, respectively. The malignant cells expressed CD20 (58%) and cyclin D1 (67%), and t(11;14) was the most common cytogenetic finding (39%). The median overall survival (OS) was 61 months. Higher ISS score was associated with worse survival (P = 0.02). Patients with IgM myeloma present with similar characteristics and outcomes as patients with more common myeloma subtypes.
IgM 型骨髓瘤是一种罕见的血液系统恶性肿瘤,其临床病理特征和患者预后尚未得到广泛研究。我们对 20 家中心的 134 例诊断为 IgM 型骨髓瘤的患者进行了多中心回顾性研究。这些患者的诊断标准为骨髓中存在 >10%的单克隆浆细胞浸润、存在任何大小的 IgM 单克隆丙种球蛋白血症、贫血、肾功能不全、高钙血症、溶骨性病变和/或通过 FISH 检测到的 t(11;14)。在这些患者中,男性多于女性(68%),中位年龄为 65.5 岁。37%的患者存在贫血,43%的患者存在肾功能不全,19%的患者存在高钙血症,70%的患者存在溶骨性病变。中位血清 IgM 水平为 2895mg/dL,19%的患者血清 IgM 水平>6000mg/dL。国际分期系统(ISS)分期为 1 期、2 期和 3 期的患者分别占 40%(33%)、54%(44%)和 29%(24%)。恶性细胞表达 CD20(58%)和 cyclin D1(67%),最常见的细胞遗传学发现是 t(11;14)(39%)。中位总生存期(OS)为 61 个月。较高的 ISS 评分与较差的生存相关(P=0.02)。IgM 型骨髓瘤患者的特征和预后与更为常见的骨髓瘤亚型相似。