Central nervous system (CNS) involvement is a rare and aggressive complication of multiple myeloma (MM). We identified 54/4352 MM patients (1.2%), who developed CNS-MM between 2000 and 2022. A matched-control group of MM patients without CNS-MM was used for comparisons. Median age was 63 years. Median time to CNS-MM was 28 months; 6/54 experienced CNS-MM at MM diagnosis. Abnormal lactate dehydrogenase (LDH), high-risk cytogenetics, and extramedullary involvement (EMI), that is, soft tissue plasmacytomas and/or plasma cell leukemia (PCL), were more frequent in CNS-MM versus controls (p < .05); 13/54 had PCL at CNS-MM. The majority had leptomeningeal infiltration (LMI) (66%); 26% had CNS-MM without systemic myeloma; EMI was the strongest predictor for CNS-MM (OR: 6.3). Median overall survival (OS) of CNS-MM patients versus controls was 43 months (95% CI: 32–54) versus 60 months (95% CI: 38–82) (p < .001); treatment of CNS-MM included mainly bortezomib/thalidomide/chemotherapy whereas 20% received novel drugs/immunotherapy combinations; 28 patients underwent cerebrospinal fluid infusions; EMI was the strongest negative predictor for post CNS-MM OS (p = .005; HR: 2.9). Treatment after 2016 predicted significantly for OS (p = .002; HR: 0.27). Median post CNS-MM OS was 4 months (95% CI: 2.6–5.4); in patients treated after 2016 median OS was 12 months. In conclusion, we have demonstrated in this large real-world series that survival of CNS-MM remains poor; however, there is a positive impact of treatment after 2016, related to the efficacy of modern anti-myeloma therapy; EMI significantly increases the probability to develop CNS-MM and the risk of post CNS-MM death, indicating a potential need for CNS prophylaxis for those patients.

中文翻译：

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中枢神经系统多发性骨髓瘤：希腊骨髓瘤研究组的真实世界多机构研究


中枢神经系统 （CNS） 受累是多发性骨髓瘤 （MM） 的一种罕见的侵袭性并发症。我们确定了 54/4352 例 MM 患者 （1.2%），他们在 2000 年至 2022 年间发生 CNS-MM。采用无 CNS-MM 的 MM 患者的匹配对照组进行比较。中位年龄为 63 岁。CNS-MM 的中位时间为 28 个月;6/54 在 MM 诊断时经历 CNS-MM。乳酸脱氢酶 （LDH） 异常、高危细胞遗传学和髓外受累 （EMI），即软组织浆细胞瘤和/或浆细胞白血病 （PCL），在 CNS-MM 中比对照组更常见 （p < .05）;13/54 在 CNS-MM 有 PCL。大多数有软脑膜浸润 （LMI） （66%）;26% 患有 CNS-MM，无系统性骨髓瘤;EMI 是 CNS-MM 的最强预测因子 （OR： 6.3）。与对照组相比，CNS-MM 患者的中位总生存期 （OS） 为 43 个月 （95% CI： 32-54） vs 60 个月 （95% CI： 38-82） （p < .001）;CNS-MM 的治疗主要包括硼替佐米/沙利度胺/化疗，而 20% 接受新药/免疫治疗联合治疗;28 例患者接受了脑脊液输注;EMI 是 CNS-MM OS 后最强的负面预测因子 （p = .005;心率：2.9）。2016 年后的治疗显着预测 OS （p = .002;心率：0.27）。CNS-MM OS 后中位为 4 个月 （95% CI： 2.6–5.4）;在 2016 年之后接受治疗的患者中，中位 OS 为 12 个月。 总之，我们已经在这个大型真实世界系列中证明，CNS-MM 的存活率仍然很差;然而，2016 年后的治疗有积极影响，与现代抗骨髓瘤治疗的疗效有关;EMI 显着增加了患 CNS-MM 的可能性和 CNS-MM 后死亡的风险，表明这些患者可能需要预防 CNS。