文章：

多发性骨髓瘤伴新发del(17p)的自然病程

Natural history of multiple myeloma with de novo del(17p)

原文发布日期：2019-03-07

DOI: 10.1038/s41408-019-0191-y

类型: Article

开放获取: 是

英文摘要：

We compared the outcomes of 310 patients with newly diagnosed multiple myeloma with del(17p) detected by FISH to patients with high-risk translocations (HRT) (n = 79) and standard-risk (SR) cytogenetics (n = 541). The median progression-free survival (PFS) following initial therapy for the three groups was 21.1, 22, and 30.1 months, respectively (P = 0.437- del(17p) vs. HRT); the median overall survival (OS) was 47.3, 79.1, and 109.8 months, respectively, (P = 0.007- del(17p) vs. HRT). PFS and OS for patients with relative loss of 17p (n = 21) were comparable to other patients with del(17p). The PFS was similar between the del(17p) and HRT groups when stratified for age, ISS stage or treatment. The OS of del(17p) and HRT groups were similar in presence of advanced age, ISS III stage or if patients did not receive a proteasome-inhibitor containing induction. ISS III stage, high LDH and HRT, but not the percentage of cells with del(17p) predicted shorter OS in patients with del(17p). The median OS for low (ISS I, normal LDH and no HRT), intermediate (neither low nor high-risk) and high-risk (ISS III and either elevated LDH or coexistent HRT) groups among del(17p) patients were 96.2, 45.4, and 22.8 months, respectively, allowing further risk stratification.
 

摘要翻译： 

我们将310例经FISH检测发现17号染色体短臂缺失（del(17p）的新诊断多发性骨髓瘤患者的结局，与高危易位（HRT）患者（n=79）和标准风险（SR）细胞遗传学患者（n=541）进行比较。三组患者初始治疗后的中位无进展生存期（PFS）分别为21.1、22和30.1个月（del(17p) vs HRT组P=0.437）；中位总生存期（OS）分别为47.3、79.1和109.8个月（del(17p) vs HRT组P=0.007）。17p相对缺失患者（n=21）的PFS和OS与其他del(17p)患者相当。按年龄、ISS分期或治疗方案分层后，del(17p)组与HRT组的PFS相近。在高龄、ISS III期或未接受含蛋白酶体抑制剂诱导治疗的情况下，del(17p)组与HRT组的OS相似。对于del(17p)患者，ISS III期、高LDH及HRT（而非del(17p)阳性细胞百分比）可预测更短的OS。根据ISS分期、LDH水平和HRT状态，将del(17p)患者进一步分为低危（ISS I、LDH正常且无HRT）、中危（非低危亦非高危）和高危（ISS III期合并LDH升高或同时存在HRT）组，其中位OS分别为96.2、45.4和22.8个月，实现了进一步的风险分层。

原文链接：

Natural history of multiple myeloma with de novo del(17p)