Malignant Haematology and Stem cell Transplantation Service, The Alfred Hospital, Melbourne, Victoria, Australia.
Br J Haematol. 2013 Aug;162(3):371-5. doi: 10.1111/bjh.12404. Epub 2013 May 29.
Central nervous system (CNS) multiple myeloma (MM) is exceedingly rare and portends a dismal prognosis. While immunomodulators have contributed to the improvement in survival in MM, they appear to have limited activity against CNS MM and, paradoxically, may contribute to the evolution of resistant MM clones capable of surviving within the CNS. We undertook a retrospective analysis to characterize the features of CNS MM and outcome in 17 patients from four institutions identified between 2000 and 2011. The median age was 58 years. Patients had received a median of three prior therapies and all had been exposed to at least one of the so-called novel anti-MM agents before the diagnosis of CNS MM. The median time to CNS disease from initial diagnosis was 36 months. Cerebrospinal fluid (CSF) light chain measurements produced discrepant results to serum light chain measurements in some patients. Treatments included systemic pharmacotherapy, intrathecal (IT) chemotherapy and/or radiotherapy (RT). The median overall survival (OS) from diagnosis of CNS MM was only 4 months. OS was significantly better in patients who received IT chemotherapy (20 months vs. 2 months, respectively; P < 0.02). We conclude that the systematic evaluation of IT therapy and diagnostic utility of CSF light chain measurements in CNS MM are warranted.
中枢神经系统（CNS）多发性骨髓瘤（MM）极为罕见，预示着预后不良。虽然免疫调节剂有助于改善 MM 的生存率，但它们似乎对 CNS-MM 的活性有限，而且矛盾的是，它们可能会导致能够在 CNS 中存活的耐药 MM 克隆的进化。我们进行了一项回顾性分析，以描述 2000 年至 2011 年间在四个机构中发现的 17 名 CNS-MM 患者的特征和结局。中位年龄为 58 岁。患者接受了中位数为三种先前治疗，所有患者在诊断 CNS-MM 之前都接受了至少一种所谓的新型抗-MM 药物治疗。从初始诊断到 CNS 疾病的中位时间为 36 个月。一些患者的脑脊液（CSF）轻链测量结果与血清轻链测量结果不一致。治疗包括全身药物治疗、鞘内（IT）化疗和/或放疗（RT）。从 CNS-MM 诊断开始的中位总生存期（OS）仅为 4 个月。接受 IT 化疗的患者 OS 明显更好（分别为 20 个月和 2 个月；P <0.02）。我们得出结论，需要对 IT 治疗进行系统评估以及 CSF 轻链测量在 CNS-MM 中的诊断实用性进行评估。
