Objective: To investigate the treatment options for multiple myeloma patients with central nervous system involvement (CNS-MM) , as well as their clinical characteristics and prognostic factors. Methods: Between January 2011 and January 2022 our center diagnosed 18 people with CNS-MM. A retrospective analysis was done on the clinical information from the initial diagnosis and central nervous system involvement, and it was compared to 1∶3 matched newly diagnosed MM from the same period. Analysis was done on the clinical characteristics and survival rates of the two groups. Results: In patients with CNS-MM, the median time of onset was 14.2 (0.9-79.6) months and the median overall survival (OS) was 30.5 months from initial diagnosis and only 3.8 months in patients after CNS involvement. The CNS-MM patients showed more IgD type (P=0.010) , severer anemia (P=0.014) , a higher proportion of bone marrow plasma cells (P=0.013) , more extramedullary lesions (P=0.001) , and increased lactic dehydrogenase (LDH) (P=0.009) when compared to the control group. Lenalidomide or pomalidomide-based combinations had higher rates of hematology and CNS remission than bortezomib or daratumumab-based regimens (75.0% vs 16.7% , P=0.019) . Patients who received IMiD-based regimens and had 2 high-risk factors at initial diagnosis (high LDH and extramedullary lesions) had a significantly lower incidence of CNS-MM (P=0.026) . At the initial diagnosis, LDH (P=0.008, HR=7.319, 95% CI 1.663-32.219) and extramedullary lesions (P=0.006, HR=8.054, 95% CI 1.828-35.486) were independent risk factors for the occurrence of CNS-MM. Conclusion: Patients with CNS-MM had a poor prognosis. Patients with high LDH or extramedullary lesions at the time of the initial diagnosis are more likely to have CNS-MM. The prognosis of this patient may be improved by immunoregulator-based therapy.
目的: 探讨累及中枢神经系统多发性骨髓瘤(CNS-MM)患者的临床特征、预后因素及治疗。 方法: 回顾性分析2011年1月至2022年1月在海军军医大学第二附属医院血液科确诊的18例CNS-MM患者在初诊及CNS受累时的临床资料,并与同时期按1∶3配对的初诊MM患者(对照组)比较,分析两组患者的临床特征、生存差异。 结果: CNS-MM自初诊时的中位发病时间为14.2(0.9~79.6)个月,中位总生存(OS)时间为30.5个月,累及CNS后的中位OS时间仅3.8个月。与对照组相比,CNS-MM患者IgD型多见(P=0.010)、贫血更严重(P=0.014)、骨髓浆细胞比例更高(P=0.013)、髓外病灶多见(P=0.001)、乳酸脱氢酶(LDH)增高(P=0.009)。基于来那度胺或泊马度胺联合方案的血液学+CNS缓解率优于硼替佐米或达雷妥尤单抗联合方案(75.0%对16.7%,P=0.019)。同时存在2个高危因素(高LDH、髓外病灶)的初诊患者,接受含免疫调节剂的方案可显著降低CNS-MM发生率(P=0.026)。初诊时高LDH(P=0.008,HR=7.319,95%CI 1.663~32.219)、存在髓外病灶(P=0.006,HR=8.054,95%CI 1.828~35.486)是发生CNS-MM的独立危险因素。 结论: CNS-MM患者预后不佳。初诊伴有高LDH或髓外病灶的患者发生CNS-MM的概率增高。基于免疫调节剂的联合治疗可能改善CNS-MM患者的预后。.
Keywords: Central nervous system; Multiple myeloma; Prognosis; Treatment.