40例多发性骨髓瘤伴髓外病变患者的临床特征分析

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个月，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％）合并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。

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

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 retrospectively 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 central 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 expected median OS was 72 months. There was a significant difference 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, Hb < 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 commonest area involved. Higher serum CRP and LDH level, more advanced stage for ISS, anemia and having EM are poor prognostic factors of MM.