多发性骨髓瘤

IF 76.9 1区 医学 Q1 MEDICINE, GENERAL & INTERNAL
Florent Malard, Paola Neri, Nizar J Bahlis, Evangelos Terpos, Nour Moukalled, Vania T M Hungria, Salomon Manier, Mohamad Mohty
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引用次数: 0

摘要

多发性骨髓瘤(MM)是一种涉及肿瘤浆细胞的血液淋巴恶性肿瘤,通常以存在单克隆免疫球蛋白蛋白为特征。骨髓瘤是第二大最常见的血液恶性肿瘤,全球发病率不断上升。由于大多数患者会复发或对治疗产生耐药性,因此该病仍无法治愈。MM是一种基因复杂的疾病,具有高度异质性,其发病过程分为多个步骤,包括肿瘤细胞的基因改变和骨髓微环境的变化。根据国际骨髓瘤工作组(International Myeloma Working Group)的标准,有症状的骨髓瘤可诊断为骨髓中克隆性浆细胞浸润≥10%,且至少存在一种骨髓瘤定义事件,即标准的CRAB特征(高钙血症、肾功能衰竭、贫血和/或溶解性骨病变)或即将发生器官损害的生物标志物。年龄较轻、身体健康的患者符合移植条件。他们先接受诱导治疗,然后用大剂量美法仑和自体造血细胞移植进行巩固治疗和维持治疗。对于老年人(不符合移植条件),首选达拉单抗、来那度胺和地塞米松联合疗法。如果复发并需要进一步治疗,将根据先前的治疗和反应选择治疗方案,目前包括免疫疗法,如双特异性单克隆抗体和嵌合抗原受体T细胞疗法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Multiple myeloma.

Multiple myeloma.

Multiple myeloma (MM) is a haematological lymphoid malignancy involving tumoural plasma cells and is usually characterized by the presence of a monoclonal immunoglobulin protein. MM is the second most common haematological malignancy, with an increasing global incidence. It remains incurable because most patients relapse or become refractory to treatments. MM is a genetically complex disease with high heterogeneity that develops as a multistep process, involving acquisition of genetic alterations in the tumour cells and changes in the bone marrow microenvironment. Symptomatic MM is diagnosed using the International Myeloma Working Group criteria as a bone marrow infiltration of ≥10% clonal plasma cells, and the presence of at least one myeloma-defining event, either standard CRAB features (hypercalcaemia, renal failure, anaemia and/or lytic bone lesions) or biomarkers of imminent organ damage. Younger and fit patients are considered eligible for transplant. They receive an induction, followed by consolidation with high-dose melphalan and autologous haematopoietic cell transplantation, and maintenance therapy. In older adults (ineligible for transplant), the combination of daratumumab, lenalidomide and dexamethasone is the preferred option. If relapse occurs and requires further therapy, the choice of therapy will be based on previous treatment and response and now includes immunotherapies, such as bi-specific monoclonal antibodies and chimeric antigen receptor T cell therapy.

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来源期刊
Nature Reviews Disease Primers
Nature Reviews Disease Primers Medicine-General Medicine
CiteScore
76.70
自引率
0.20%
发文量
75
期刊介绍: Nature Reviews Disease Primers, a part of the Nature Reviews journal portfolio, features sections on epidemiology, mechanisms, diagnosis, management, and patient quality of life. The editorial team commissions top researchers — comprising basic scientists and clinical researchers — to write the Primers, which are designed for use by early career researchers, medical students and principal investigators. Each Primer concludes with an Outlook section, highlighting future research directions. Covered medical specialties include Cardiology, Dermatology, Ear, Nose and Throat, Emergency Medicine, Endocrinology, Gastroenterology, Genetic Conditions, Gynaecology and Obstetrics, Hepatology, Haematology, Infectious Diseases, Maxillofacial and Oral Medicine, Nephrology, Neurology, Nutrition, Oncology, Ophthalmology, Orthopaedics, Psychiatry, Respiratory Medicine, Rheumatology, Sleep Medicine, and Urology.
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