IgD型多发性骨髓瘤患者临床特征及生存分析  

Clinical Characteristics and Survival Analysis of Patients with IgD Multiple Myeloma

作　　者：高晓云 马艳萍[1] 晁瑶 范丽 GAO Xiao-Yun;MA Yan-Ping;CHAO Yao;FAN Li(Department of Hematology,The Second Hospital of Shanxi Medical University,Taiyuan 030001,Shanxi Province,China)

机构地区：[1]山西医科大学第二医院血液科,山西太原030001

出　　处：《中国实验血液学杂志》2021年第2期547-552,共6页Journal of Experimental Hematology

基　　金：山西省重点研发计划项目(No.201903D321099)。

摘　　要：目的:探讨Ig D型多发性骨髓瘤(MM)患者的临床特征、预后及生存。方法:回顾性分析20例Ig D型MM患者临床资料,总结其临床特征,分析预后因素及生存。采用Kaplan-Meier方法进行生存分析,预后因素分析应用Log-rank检验,对观察指标进行单因素分析,单因素分析中P<0.15的变量纳入多因素COX回归分析。结果:Ig D型MM患者占同期全部MM患者的4.3%,其中男性多见(占80%),中位发病年龄57.5(35-77)岁,以λ轻链型为主,占90%。DS-Ⅲ期18例(90%),ISS-Ⅲ期10例(50%),首发临床表现主要有乏力、骨痛、肾功能损害,贫血(血红蛋白<100 g/L)14例(70%),溶骨性骨质破坏≥3处者12例(60%),合并肾功能损害者8例(40%),血钙升高者11例(51.4%)。仅5例患者白球比例倒置,低白蛋白血症占40%,球蛋白升高者仅占15%。FISH结果显示,1q21扩增阳性发生率最高,占50%,且易与其他细胞遗传学异常同时出现。发生髓外浸润者4例(20%)。预后因素分析显示,仅乳酸脱氢酶水平升高是Ig D型MM患者独立预后不良因素。2例原发耐药患者均存在髓外浸润及多种细胞遗传学异常,提示髓外浸润及多种细胞遗传学异常可能为预后不良因素,但差异不具有统计学意义,可能与样本量少有关。20例患者均使用含硼替佐米方案治疗,可评价疗效的有19例,17例(89.4%)患者治疗有效,其中CR+VGPR占52.6%、PR患者6例(31.5%)、MR患者1例(5.3%),2例患者原发耐药。中位无进展生存时间及总生存时间分别为9.5和20.5个月。结论:Ig D型MM是一种少见且侵袭性较强的疾病,乳酸脱氢酶水平升高是这类患者的独立预后不良因素,含硼替佐米方案可改善Ig D型MM患者的预后。Objective:To explore the clinical features,prognosis and survival of patients with IgD multiple myeloma(MM).Methods:The clinical data of 20 patients with IgD MM was analyzed retrospectively.The prognostic factors and survival analysis was carried out.We summarized their clinical characteristics.The survival analysis was carried out by Kaplan-Meier method,and the prognostic factor were analyzed by using log-rank test for single factor analysis of observation index.Variables of P<0.15 in single factor analysis were enrolled in multifactor cox regression analysis.Results:IgD MM patients accounted for 4.3%of all MM patients in the same period,among which 80%were male,the median age of patients was 57.5(35-77)years old,90%of the patients belongs toλlight chain type.At the time of diagnosis,18 patients(90%)were in DS-III stages,while 10 patients were in ISS-III stage.The first clinical manifestations were fatigue,bone pain,kidney function impairment,anemia(Hb<100 g/L)in 14 cases(70%),12 cases(60%)with osteolytic bone destruction≥3,combined with renal impairment in 8 cases(40%),and elevated blood calcium in 11 cases(51.4%).In only 5 patients the ratio of albumin to globntin was inverted,hypoalbuminemia accounted for 40%,and globulin increase accounted for only 15%.FISH results showed that the positive rate of 1 q21 amplification(50%)was the highest,and it was easy to occur at the same time as other cytogenetic abnormalities.Extramedullary infiltration occurred in 4 cases(20%).The analysis of prognostic factors showed that only the increase of lactate dehydrogenase(LDH)level was an independent poor prognostic factor for IgD MM patients.Extramedullary infiltration and various cytogenetic abnormalities were found in 2 IgD MM patients with primary drug resistance,suggesting that extramedullary infiltration and various cytogenetic abnormalities may be prognostic factors,but the difference was not statistically significant,Which maybe related to the small sample size.All 20 patients were treated with bortezomib-containing

关 键 词：多发性骨髓瘤 免疫球蛋白D 临床特征 预后 硼替佐米 

分 类 号：R733.3[医药卫生—肿瘤]