| 李 新,黄仲夏.多发性骨髓瘤伴髓外病变研究进展[J].肿瘤学杂志,2020,26(2):2-86. |
| 多发性骨髓瘤伴髓外病变研究进展 |
| Research Progress on Multiple Myeloma with Extramedullary Disease |
| 投稿时间：2019-05-22 |
| DOI：10.11735/j.issn.1671-170X.2020.02.B002 |
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| 中文关键词: 多发性骨髓瘤 髓外病变 预后 治疗 |
| 英文关键词:multiple myeloma extramedullary disease prognosis treatment |
| 基金项目:北京市科技计划课题（Z171100000417010)；北京市石景山区医学重点扶持专科建设项目 (20170006) |
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| 中文摘要: |
| 摘 要：多发性骨髓瘤（MM）伴髓外病变（EMD）的发病率和发现率逐年升高。EMD包括三种类型：①骨髓瘤侵及邻近骨皮质并通过局部生长形成软组织包块；②不与骨相连的软组织肿块或内脏肿块，通过血行转移至骨髓远处器官或组织，表现为单一或多发的的结节或肿块；③骨髓瘤细胞弥漫浸润器官而没有局灶性病变，如胸水等。骨髓微环境通过引起信号分子级联反应介导骨髓瘤细胞增殖、转移、存活，促使骨髓瘤生长和恶性浆细胞归巢。伴EMD的MM患者总体生存期短、预后差。治疗上可选择蛋白酶体抑制剂联合IMiDs，VTD/-PACE、单抗Dara、elotuzumab、抗BCMA、BCL-2靶向药物等，也可以考虑allo-SCT或序贯ASCT。 |
| 英文摘要: |
| Abstract：The incidence of multiple myeloma(MM)with extramedullary disease(EMD)has increased. There are three types of EMD clinically：①Tumor mass adjacent to bone and extending into soft tissue.②A soft tissue mass or visceral mass not connecting to the bone；and metastasis to distant organs or tissues of the bone marrow by blood，manifesting as a single or multiple nodule or mass.③ Myeloma cells diffuse into organs without focal lesions，such as pleural effusion. The bone morrow microenvironment plays a key role in the proliferation，transfer，homing and survival of plasmocyte. The survival time of patients with EMD is short and the prognosis is poor. Proteasome inhibitors combined with IMiDs，VTD/-PACE，monoclonal antibodies Dara，elotuzumab，anti-BCMA，BCL-2 targeted drugs can be selected for treatment，allo-SCT or sequential ASCT can also be considered. |
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