130例IgD型多发性骨髓瘤临床特点及预后分析

Clinical characteristics and prognosis analysis of 130 cases with IgD multiple myeloma

目的:分析IgD型多发性骨髓瘤(MM)患者的临床特点、疗效及预后因素。方法:回顾性分析130例IgD型MM患者的病例资料,对其临床特征、实验室指标、生存和预后因素进行分析。结果:130例IgD型MM患者,中位发病年龄为55.5(33~83)岁,男女比例为2.3∶1.0,λ轻链型123例(占94.6%);103例患者有可分析的FISH结果,17p缺失、13q缺失、1q21扩增、t(11;14)、t(4;14)的发生率分别为15.5%(16/103)、28.2%(29/103)、68.0%(70/103)、45.5%(40/88)、4.5%(4/88),其中70例1q21扩增患者中,30例合并t(11;14)(其中9例同时伴13q缺失),12例合并17p缺失,19例合并13q缺失。22例采用传统化疗方案诱导治疗,108例采用以硼替佐米为基础的联合化疗方案诱导治疗,2组的总体反应率分别为63.6%和86.1%(P=0.012)。130例患者中位随访时间24.3个月,中位生存期为31.1个月,多因素分析结果显示,乳酸脱氢酶(LDH)是IgD型MM的独立预后因素,LDH升高的患者中位生存期为21.6个月,LDH正常的患者中位生存期为39.5个月,2组差异有统计学意义(P<0.000 1)。结论:IgD型MM是一种少见的MM类型,发病年龄较年轻,以λ轻链型为主,预后较差;FISH检测1q21扩增发生率较高,常合并其他遗传学异常,可能提示预后不良;采用新药化疗可提高其总体反应率;LDH是IgD型MM的独立预后因素。

Objective： To evaluate the clinical characteristics, curative efficacy and prognosis of IgD multiple myeloma （MM）. Method：The clinical characteristics, curative efficacy, survival and prognosis of 130 cases of IgD MM were retrospectively analyzed. Result：The median age of 130 cases of IgD MM was 55.5 years （range 33 to 83） ,the ratio of mate to female was 2.3 = 1.0,k hght chain accounted for 94.6% （123/130）. One hundred and three patients achieved FISH, the positive rates of 17p deletion, 13q deletion, lq21 amplification, t（ 11 ; 14）, t（4 ; 14） were 15.5%（16/103）,28.2% （29/103）,68.0% （70/103）, 45. 5% （40/88）,4.5% （4/88）,respectively. In 70 patients with lq21 amplification,a0 cases complicated with t（11;14） （9 cases also had 18q deletion）, 12 patients complicated with 17p deletion, 19 patients complicated with 13q deletion. The overall response rate was 63.60//00 in traditional chemotherapy group （n=29,） and 86.1% in bortezomib based combination chemotherapy group （n = 108） （P=0. 012）. The median follow-up time was 24.3 months and the median overall survival time was 31.1 months in 130 IgD MM patients. Multivariate analysis showed that LDH was independently adverse prognosis factor. The median overall survival time was 21.6 months vs. 39.5 months in high-LDH and normal LDH, respectively, with statistical significance （P（0. 0001）. Conclusion： IgD MM is a rare type of MM, which occurred in younger age. k is the main type of light chain. The IgD MM patients demostrated high positive rate of lq21 amplification and often combined with other cytogenetic aberrations,which may indicate poor prognosis. The use of new drugs have improved the overall response rate in IgD MM. LDH is independently adverse factor affecting IgD MM prognosis.