Objective: To compare the clinical characteristics and outcomes of patients newly diagnosed with multiple myeloma(NDMM)with bone-related extramedullary(EM-B)disease and those with extraosseous extramedullary(EM-E)disease and to address their prognostic factors. Methods: The clinical features, outcomes, and prognostic factors were retrospectively analyzed in 80 patients with NDMM with extramedullary disease. Results: Among 80 patients with extramedullary disease, 51 had EM-B and 29 EM-E. The level of β(2)-microglobulin(5.82 mg/L vs 3.99 mg/L, P=0.030), lactate dehydrogenase(256 U/L vs 184 U/L, P=0.003), 1q21 amplification rate(78.6% vs 53.1%, P=0.035), and Ki-67 proliferation index(50% vs 25%, P=0.002)in the EME group were significantly higher than those in the EM-B group. The posieive rate of CD56(14.3% vs 66.7%)and overall response rate(60% vs 82.3%)in EM-E group were significantly lower than those in EM-B group. The median overall survival (OS)of patients with EM-E and EM-B was 14.5 and 49.5 months, and the median progression-free survival(PFS)of the two groups was 9.0 and 18.0 months. Patients with EM-E had a significantly shorter OS(P=0.035)and PFS(P < 0.001)than those of patients with EM-B, whereas the PFS did not significantly differ(P=0.263)when patients accepted proteasome inhibitor(PI)-based regimens for induction therapy. Multivariate analysis with Cox model showed the best response that did not achieve partial response(PR)was an independent poor prognostic factor for both OS and PFS in NDMM patients with EM-E(P=0.031, P=0.005), ISS-III, and the best response that did not achieve PR were independent prognostic factors for the shorter OS in patients with NDMM with EM-B(P=0.009, P=0.044). Conclusions: The clinical characteristics and outcomes of patients with NDMM with EM-E are different from patients with EM-B. Outcomes of patients with EM-E is significantly poor. PI induction therapy improved the PFS of patients with EM-E.
目的: 比较伴骨相关髓外(EM-B)和骨外髓外(EM-E)病变的初诊多发性骨髓瘤(NDMM)患者的临床特征及预后,并探讨影响预后的因素。 方法: 回顾性分析2009年11月至2019年3月江苏省人民医院血液科诊治的80例伴髓外病变的NDMM患者的临床特征、预后及影响预后的因素。 结果: 80例累及髓外的NDMM患者中伴EM-B者51例,伴EM-E者29例。与EM-B组相比,EM-E组β(2)-微球蛋白水平(5.82 mg/L对3.99 mg/L,P=0.030)、乳酸脱氢酶水平(256 U/L对184 U/L,P=0.003)、1q21扩增发生率(78.6%对53.1%,P=0.035)、肿瘤细胞Ki-67增殖指数(50%对25%,P=0.002)升高,CD56阳性率(14.3%对66.7%,P=0.057)和治疗总有效率(60.0%对82.3%,P=0.034)降低。EM-E组和EM-B组的中位总生存(OS)时间分别为14.5、49.5个月,中位无进展生存(PFS)时间分别为9.0、18.0个月。与EM-B组患者相比,EM-E组患者的OS、PFS时间均明显缩短(P值分别为0.035和<0.001)。在接受蛋白酶体抑制剂诱导化疗的患者中,两组PFS时间的差异无统计学意义(P=0.263)。Cox回归多因素分析显示:诱导治疗后最佳疗效未达部分缓解(PR)是EM-E组患者OS、PFS的独立不良预后因素(P值分别为0.031、0.005);ISS-Ⅲ期、诱导治疗后最佳疗效未达PR为EM-B组患者OS的独立不良预后因素(P值分别为0.009、0.044)。 结论: 伴EM-E的NDMM患者与伴EM-B的患者有不同的临床特征及预后,前者的预后明显较后者差,蛋白酶体抑制剂可改善EM-E组患者的PFS。.
Keywords: Clinical features; Extramedullary disease; Multiple myeloma; Prognosis.