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累及中枢神经系统的多发性骨髓瘤患者临床特征分析

Clinical characteristics of patients with multiple myeloma involving central nervous system
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摘要 目的探讨累及中枢神经系统(CNS)多发性骨髓瘤(MM)患者的临床特征、治疗方案及预后影响因素。方法回顾性分析2014年1月至2020年6月海军军医大学第二附属医院收治的18例累及CNS的MM患者临床资料, 分析其临床特征、治疗情况及预后;采用Kaplan-Meier法进行生存分析, 并进行log-rank检验;采用Cox比例风险模型进行单因素和多因素分析。结果 18例患者中, 男性12例, 女性6例;累及CNS时中位年龄54岁(38~71岁)。MM从诊断至累及CNS的中位时间为22个月(0~126个月)。原发性CNS受累1例, 继发性CNS受累17例。Durie-Salmon(DS)分期均为Ⅲ期;国际预后分期系统(ISS)分期Ⅲ期10例, Ⅱ期6例, Ⅰ期2例。CNS受累部位:单独累及硬脑膜7例, 单独累及软脑膜4例, 单独累及脑实质2例, 同时累及脑膜及脑实质5例。最常见的神经系统症状为头痛、颅神经麻痹, 9例患者出现多种神经系统症状。所有患者均接受全身系统治疗, 16例患者接受鞘内注射和(或)放疗;总有效率为66.7%, 其中最佳疗效达到严格意义的完全缓解(sCR)3例、完全缓解(CR)1例、非常好的部分缓解(VGPR)3例、部分缓解(PR)5例。18例患者中位总生存(OS)时间32.7个月;从累及CNS后计算, 中位无进展生存(PFS)时间7.5个月, 中位OS时间12.2个月;单独累及硬脑膜组中位PFS时间长于非单独累及硬脑膜组(15.1个月比5.9个月, P=0.009);单独累及硬脑膜组中位OS时间长于非单独累及硬脑膜组(16.9个月比10.7个月, P=0.175)。是否单独累及硬脑膜是影响累及CNS的MM患者PFS的独立影响因素(HR=0.191, 95%CI 0.038~0.952, P=0.043)。结论累及CNS的MM患者罕见, 预后差, CNS受累部位影响患者的预后, 目前尚缺乏有效的治疗手段。 Objective To explore the clinical characteristics,treatment regimens and prognostic influencing factors of patients with multiple myeloma(MM)involving central nervous system(CNS).Methods The clinical data of 18 MM patients involving CNS in Second Affiliated Hospital of Naval Medical University from January 2014 to June 2020 were retrospectively analyzed.Their clinical characteristics,treatment and prognosis were also analyzed.Kaplan-Meier method was used to make survival analysis and log-rank was performed;Cox proportional risk model was used to make univariate and multivariate analysis.Results The cohort of 18 patients included 12 males and 6 females;the median age of patients involving CNS was 54 years(38-71 years).The median time from diagnosis to the involvement of CNS was 22 months(0-126 months).Among 18 patients,1 case was primary MM involving CNS,and 17 cases were secondary MM involving CNS.All patients had Durie-Salmon(DS)stageⅢ;10 cases had international staging system(ISS)stageⅢ,6 cases had ISS stageⅡ,and 2 cases had ISS stageⅠ.Involvement sites of CNS included 7 cases of involving the dura mater alone and 4 cases of involving the pia mater alone,2 cases of involving brain parenchyma and 5 cases of involving both meninges and brain parenchyma.The most common neurological symptoms were headache and cranial nerve palsy,and 9 patients had multiple neurological symptoms.All patients received systemic therapy,16 patients received an intrathecal injection and/or radiotherapy;and the overall effective rate was 66.7%,including 3 achieving strict complete remission(sCR),1 achieving complete remission(CR),3 achieving very good partial remission(VGPR),5 achieving partial remission(PR).The median overall survival(OS)was 32.7 months.Counting from the point of CNS involvement,the median progression-free survival(PFS)and OS time was 7.5 months,12.2 months,respectively.The median PFS of MM patients in the dura-involved alone group was longer than that in the non-dura-involved alone group(15.1 months vs.5.9 mont
作者 强琬婷 刘进 卢静 何海燕 杜鹃 Qiang Wanting;Liu Jin;Lu Jing;He Haiyan;Du Juan(Department of Hematology,Second Affiliated Hospital of Naval Medical University,Shanghai 200003,China)
出处 《白血病.淋巴瘤》 CAS 2023年第6期329-334,共6页 Journal of Leukemia & Lymphoma
关键词 多发性骨髓瘤 中枢神经系统 预后 Multiple myeloma Central nervous system Prognosis
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