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目的 :探讨免疫球蛋白D(IgD)型多发性骨髓瘤(MM)患者的临床特点和疗效。方法:回顾性分析343例初诊骨髓瘤患者中23例(7%)IgD型骨髓瘤患者的临床特点,比较传统治疗、新药及自体干细胞移植对生存的影响。结果:IgD型患者发病中位年龄为58(41~75)岁,λ型占91.3%,Durie-SalmonⅢ期19例,国际分期体系(ISS)Ⅲ期14例。骨损害18例(78%),髓外累及5例(22%),肾功能受损9例(39%),贫血20例(87%)。诱导后传统化学治疗(化疗)组(6例)和新药(硼替佐米或沙利度胺)治疗组(17例)总反应率分别为67%和100%。中位随访31(18~71)个月,比较传统组与新药组,中位无进展生存(PFS)期分别为(16.0±9.5)个月和(20.2±1.9)个月(P=0.397),中位总生存(OS)期2组分别为(39.3±11.1)个月和(55.5±7.8)个月(P=0.231)。比较移植组(6例)和非移植组(17例),中位OS期分别为(69.2±10.2)个月和(44.2±6.6)个月(P=0.059)。结论:IgD型骨髓瘤常伴有不良临床特征,新药应用能提高缓解率和缓解程度,自体干细胞移植有进一步延长生存的趋势。
Objective: To investigate the clinical features and efficacy of immunoglobulin D (IgD) multiple myeloma (MM). Methods: The clinical features of 23 patients (7%) with IgD myeloma from 343 newly diagnosed myeloma patients were retrospectively analyzed. The effects of traditional therapy, new drugs and autologous stem cell transplantation on survival were compared. Results: The median age of patients with IgD was 58 (41-75) years old, λ type accounted for 91.3%, Durie-Salmon Ⅲ 19 cases, and International Staging System Ⅲ (ISS Ⅲ) 14 cases. Bone damage was found in 18 patients (78%), extramedullary involvement in 5 patients (22%), impaired renal function in 9 patients (39%) and anemia in 20 patients (87%). After induction, the overall response rate was 67% and 100% for the traditional chemotherapy (6 cases) group and the new drug (bortezomib or thalidomide) treatment group (17 cases), respectively. The median follow-up was 31 months (18-71). The median progression-free survival (PFS) was (16.0 ± 9.5) months and (20.2 ± 1.9) months respectively in the traditional and new drug groups (P = 0.397) (39.3 ± 11.1) months and (55.5 ± 7.8) months, respectively (P = 0.231) in the median OS. The median OS was (69.2 ± 10.2) months and (44.2 ± 6.6) months, respectively (P = 0.059) compared with the transplantation group (6 cases) and the non-transplantation group (17 cases). Conclusion: IgD type myeloma often associated with poor clinical features, application of new drugs can improve the remission rate and degree of remission, autologous stem cell transplantation to further extend the survival trend.