| 胡婉莉,宋佳殷,魏宇君.多发性骨髓瘤合并中枢神经系统髓外病变诊治进展[J].肿瘤学杂志,2021,27(12):1028-1033. |
| 多发性骨髓瘤合并中枢神经系统髓外病变诊治进展 |
| Progress on Diagnosis and Treatment of Multiple Myeloma with Extramedullary Disease of Central Nervous System |
| 中文关键词 |
| DOI：10.11735/j.issn.1671-170X.2021.12.B011 |
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| 中文关键词: 多发性骨髓瘤 髓外病变 中枢神经系统 诊断 治疗 预后 |
| 英文关键词:multiple myeloma extramedullary disease central nervous system diagnosis treatment prognosis |
| 基金项目:北京市科技计划（Z171100000417010）；北京市石景山区医学重点专科建设项目 |
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| 中文摘要: |
| 摘 要：多发性骨髓瘤（multiple myeloma，MM）是一种起源于骨髓的恶性浆细胞肿瘤，新诊断MM或复发难治MM均可合并髓外病变。其中，MM合并中枢神经系统髓外病变的发病率约1%，患者通常表现为局灶性神经功能缺损、视力改变、神经根病、头痛、精神状态改变、认知障碍症状等。增强磁共振成像是标准放射学检查方法，同时脑组织或脑脊液检查中可见单克隆浆细胞有助于诊断。治疗上，目前以蛋白酶体抑制剂、免疫调节剂等新药化疗、自体干细胞移植和放疗相结合的积极治疗策略，但MM合并髓外病变患者的预后仍较差，中位生存期仅6～10个月。 |
| 英文摘要: |
| Abstract： Multiple myeloma(MM) is a malignant plasma cell tumor originating from the bone marrow， and can be combined with extramedullary disease(EMD) in either newly diagnosed MM(NDMM) or relapsed refractory MM(RRMM). The incidence of MM combined with extramedullary disease of central nervous system(CNS) is about 1%， and patients usually present with focal neurological deficits， visual changes， radiculopathy， headaches， changes in mental status， and symptoms of cognitive impairment. Enhanced MRI is a standard radiological examination method， meanwhile， monoclonal plasma cells detected with cerebrospinal fluid examination would help the diagnosis. The comprehensive treatment strategy， which combines chemotherapy with proteasome inhibitors and immunomodulators， autologous stem cell transplantation， and radiotherapy is generally adopted， the prognosis of these patients is still poor， with an average median survival time of only 6～10 months. |
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