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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 analyze the clinical features, efficacy and prognosis of patients with IgD multiple myeloma (MM). Methods: A retrospective analysis of 130 cases of IgD MM patients, clinical data, laboratory parameters, survival and prognostic factors were analyzed. Results: The median age at onset of IgM MM was 55.5 (33-83) years old, with a ratio of 2.3:1.0 for males to females and 123 cases for λ light chain (94.6%). There were 103 FISH results that were analyzable , 17p deletion, 13q deletion, 1q21 amplification, t (11,14) and t (4,14) were 15.5% (16/103), 28.2% (29/103) and 68.0% 103), 45.5% (40/88) and 4.5% (4/88). Of the 70 patients with 1q21 amplification, 30 had t (11; 14) (9 of them were accompanied by 13q deletion) 17p deletion, 19 cases combined with 13q deletion. Twenty-two patients were induced by traditional chemotherapy and 108 were treated with bortezomib-based combination chemotherapy. The overall response rates in two groups were 63.6% and 86.1%, respectively (P = 0.012). 130 patients with a median follow-up time of 24.3 months, a median survival of 31.1 months, multivariate analysis showed that lactate dehydrogenase (LDH) is an independent prognostic factor IgD MM, elevated median survival in patients with elevated LDH The median survival time was 21.6 months in patients with normal LDH, and 39.5 months in patients with normal LDH. The difference between the two groups was statistically significant (P <0.000 1). Conclusion: IgD type MM is a rare type of MM, the age of onset is younger, with λ light chain type, the prognosis is poor; FISH detection of 1q21 amplification rate is higher, often associated with other genetic abnormalities may prompt the prognosis Poor; using new drug chemotherapy can improve its overall response rate; LDH IgD MM is an independent prognostic factor.