40例多发性骨髓瘤伴髓外病变患者的临床特征分析

Clinical features of multiple myeloma patients with extramedullary disease:a report of 40 cases from a single center

目的分析多发性骨髓瘤(MM)患者在初诊时或诊治病程中并发髓外病变(EM)的临床特征和危险因素。方法回顾性分析1993至2006年在我院住院治疗的418例 MM 患者中并发 EM的临床特征、生存率以及预后因素。结果 418例 MM 患者中,40例(9.6%)并发 EM,常见受累部位前三位依次为软组织、胸(腹)膜和中枢神经系统;中位随访30个月,中位总体生存(OS)时间为28个门,初诊时25例(6%)合并 EM,初诊合并 EM组(A 组)中位 OS 时间仅为16个月,病程中有15例(3.6%)合行 EM,病程中合并 EM 组(B 组)估计中位 OS 时间为72个月,两组相比差异具有统计学意义(P=0.0045);与 A 组相比较,B 组患者初诊时骨髓浆细胞(P=0.022)及骨髓幼稚浆细胞比例(原始、幼稚浆细胞占所有浆细胞比例)(P=0.029)更高,ISS 分期较早(P=0.027)。单因素分析 MM 并发 EM 患者初诊时 C 反应蛋白(CRP)水平增高,血清乳酸脱氢酶(LDH)增高,ISS 分期为Ⅱ、Ⅲ期,Hb<110g/L。初诊时合并 EM 的 MM 预后不良,但 COX 多因素分析未显示统计学差异。结论 MM合并 EM 并不少见,最常见受累部位为软组织。CRP 增高、LDH 升高、ISS 分期晚、贫血及 EM 是 MM 不良预后指标,MM 患者并发 EM 后临床缺乏有效的治疗手段。

Objective To analyze the clinical and laboratory features and risk factors of multiple myeloma（MM） with extramedullary disease （EM） and its extraosseous localizations at diagnosis and during the course of MM. Methods The clinical features, survival rate and prognostic factors were retrospeetively analyzed in 40 patients having EM from a total of 418 MM patients hospitalized in Changzheng Hospital from 1993 to 2006. Results Among the 40 patients, the first three localizations of EM involved soft tissue, pleura or peritoneum and eentral nervous system （CNS）. Median duration of follow-up was 30 months. The median overall survival （OS） was 28 months. Twenty-five patients （6%） were found to have EM at diagnosis （ group A） , and their median OS was 16 months and 15 patients （3.6%） developed EM during the course of the disease （group B） , and their expeeted median OS was 72 months. There was a significant differenee between group A and B （ P = 0.0045） for OS. Compared with those in group A, patients in group B had a higher percentage of plasmacytes （ P = 0.022） and plasmablasts （ P = 0.029） in bone marrow, and less advanced stage for international staging system （ISS） （P =0.027）. Log-rank univariate analysis showed that higher CRP level, higher serum LDH, Stage Ⅱ and Ⅲ for ISS, lib 〈 110 g/L at diagnosis were poor prognostic factors. However, multivariate analysis with COX model showed none of them were statistically significant. Conclusion EM tumors are not a rare manifestation of MM. Soft tissue in the eommonest area involved. Higher serum CRP and LDli level, more advaneed stage for ISS, anemia and having EM are poor prognostie faetors of MM.