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多发性骨髓瘤的流行病学、分期和治疗。

Epidemiology, Staging, and Management of Multiple Myeloma.

机构信息

Department of Medicine, Division of Nephrology, Hypertension and Transplant, Medical College of Georgia, Augusta University, Augusta, GA 30912, USA.

Sidney Kimmel Cancer Center, Jefferson University, Philadelphia, PA 19107, USA.

出版信息

Med Sci (Basel). 2021 Jan 20;9(1):3. doi: 10.3390/medsci9010003.

DOI:10.3390/medsci9010003
PMID:33498356
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7838784/
Abstract

Multiple myeloma (MM) is a plasma cell disorder that is on the rise throughout the world, especially in the US, Australia, and Western Europe. In the US, MM accounts for almost 2% of cancer diagnoses and over 2% of cancer deaths (more than double the global proportion). Incidence has risen by 126% globally and over 40% in the US since 1990, while global mortality has risen by 94% and US mortality has fallen by 18%. The 5 year survival in the US has more than doubled over the past decades with the introduction of new targeted therapies and transplant techniques. Risk factors for MM include age (average age of diagnosis is 69), race (African Americans are over double as likely to be diagnosed), sex (men are at a 1.5× risk), and family history. Diagnosis includes serum or urine electrophoresis and free light-chain assay but requires bone marrow biopsy. It is distinguished from smoldering myeloma and monoclonal gammopathy of undetermined significance by a high (>3 g/dL) level of M-protein (monoclonal light chains) and the presence of CRAB (Hypercalcemia, Renal failure, Anemia, Bone pain) symptoms, which include hypercalcemia, renal failure, anemia, and bone pain, suggesting an end-organ damage. International staging system staging involves beta 2 microglobulin and albumin levels, while the revised system considers prognostic factors such as lactate dehydrogenase levels and chromosomal abnormalities. Front-line management includes induction regimen, maintenance therapy and hematopoietic cell transplantation for eligible patients and bisphosphonates or bone-stimulating agents for the prevention of skeletal events. Treatment for relapsed disease includes newly approved monoclonal antibodies like the CD38-targeting daratumumab, proteasome inhibitors, immunomodulating agents, and investigational therapies such as B cell maturation antigen Chimeric antigen receptor T cells.

摘要

多发性骨髓瘤(MM)是一种浆细胞疾病,在全球范围内呈上升趋势,尤其在美国、澳大利亚和西欧。在美国,MM 约占癌症诊断的 2%,癌症死亡人数的 2%以上(是全球比例的两倍多)。自 1990 年以来,全球发病率上升了 126%,美国上升了 40%以上,而全球死亡率上升了 94%,美国死亡率下降了 18%。在过去几十年中,随着新的靶向治疗和移植技术的引入,美国的 5 年生存率增加了一倍以上。MM 的危险因素包括年龄(诊断平均年龄为 69 岁)、种族(非裔美国人的诊断可能性是其两倍多)、性别(男性的风险是 1.5 倍)和家族史。诊断包括血清或尿液电泳和游离轻链检测,但需要骨髓活检。它与冒烟型骨髓瘤和意义未明的单克隆丙种球蛋白病不同,其特点是 M 蛋白(单克隆轻链)水平较高(>3g/dL),并存在 CRAB(高钙血症、肾衰竭、贫血、骨痛)症状,包括高钙血症、肾衰竭、贫血和骨痛,提示存在终末器官损伤。国际分期系统分期涉及β2 微球蛋白和白蛋白水平,而修订后的系统则考虑了乳酸脱氢酶水平和染色体异常等预后因素。一线治疗包括适合患者的诱导方案、维持治疗和造血细胞移植,以及双膦酸盐或骨刺激剂预防骨骼事件。复发性疾病的治疗包括新批准的单克隆抗体,如靶向 CD38 的达雷妥尤单抗、蛋白酶体抑制剂、免疫调节剂和研究性疗法,如 B 细胞成熟抗原嵌合抗原受体 T 细胞。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f829/7838784/ba11151ea666/medsci-09-00003-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f829/7838784/27e6ed5cd7a7/medsci-09-00003-g001.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f829/7838784/cf8274c0bdc9/medsci-09-00003-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f829/7838784/0c709972fa70/medsci-09-00003-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f829/7838784/ba11151ea666/medsci-09-00003-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f829/7838784/27e6ed5cd7a7/medsci-09-00003-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f829/7838784/63497c65bc9b/medsci-09-00003-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f829/7838784/a30b9b56f1e5/medsci-09-00003-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f829/7838784/91be1fd896ae/medsci-09-00003-g004.jpg
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