Mesh : Aged Biomarkers / blood Consolidation Chemotherapy / methods Disease Management Hematopoietic Stem Cell Transplantation Humans In Situ Hybridization, Fluorescence Induction Chemotherapy / methods Maintenance Chemotherapy / methods Multiple Myeloma / complications diagnosis genetics therapy Progression-Free Survival Recurrence Retreatment / methods Transplantation, Autologous

来  源:   DOI:10.1001/jama.2022.0003

Abstract:
OBJECTIVE: Multiple myeloma is a hematologic malignancy characterized by presence of abnormal clonal plasma cells in the bone marrow, with potential for uncontrolled growth causing destructive bone lesions, kidney injury, anemia, and hypercalcemia. Multiple myeloma is diagnosed in an estimated 34 920 people in the US and in approximately 588 161 people worldwide each year.
METHODS: Among patients with multiple myeloma, approximately 73% have anemia, 79% have osteolytic bone disease, and 19% have acute kidney injury at the time of presentation. Evaluation of patients with possible multiple myeloma includes measurement of hemoglobin, serum creatinine, serum calcium, and serum free light chain levels; serum protein electrophoresis with immunofixation; 24-hour urine protein electrophoresis; and full-body skeletal imaging with computed tomography, positron emission tomography, or magnetic resonance imaging. The Revised International Staging System combines data from the serum biomarkers β2 microglobulin, albumin, and lactate dehydrogenase in conjunction with malignant plasma cell genomic features found on fluorescence in situ hybridization-t(4;14), del(17p), and t(14;16)-to assess estimated progression-free survival and overall survival. At diagnosis, 28% of patients are classified as having Revised International Staging stage I multiple myeloma, and these patients have a median 5-year survival of 82%. Among all patients with multiple myeloma, standard first-line (induction) therapy consists of a combination of an injectable proteasome inhibitor (ie, bortezomib), an oral immunomodulatory agent (ie, lenalidomide), and dexamethasone and is associated with median progression-free survival of 41 months, compared with historical reports of 8.5 months without therapy. This induction therapy combined with autologous hematopoietic stem cell transplantation followed by maintenance lenalidomide is standard of care for eligible patients.
CONCLUSIONS: Approximately 34 920 people in the US and 155 688 people worldwide are diagnosed with multiple myeloma each year. Induction therapy with an injectable proteasome inhibitor, an oral immunomodulatory agent and dexamethasone followed by treatment with autologous hematopoietic stem cell transplantation, and maintenance therapy with lenalidomide are among the treatments considered standard care for eligible patients.
摘要:
目的:多发性骨髓瘤是一种血液系统恶性肿瘤,其特征是骨髓中存在异常克隆性浆细胞,有可能不受控制的生长导致破坏性的骨损伤,肾损伤,贫血,和高钙血症。在美国,估计有34920人被诊断出多发性骨髓瘤,全世界每年约有588161人被诊断出多发性骨髓瘤。
方法:在多发性骨髓瘤患者中,大约73%有贫血,79%有溶骨病,19%的患者在就诊时出现急性肾损伤。对可能患有多发性骨髓瘤的患者的评估包括测量血红蛋白,血清肌酐,血清钙,和血清游离轻链水平;血清蛋白电泳与免疫固定;24小时尿蛋白电泳;和全身骨骼成像与计算机断层扫描,正电子发射断层扫描,或者磁共振成像.修订后的国际分期系统结合了血清生物标志物β2微球蛋白的数据,白蛋白,和乳酸脱氢酶结合在荧光原位杂交-t(4;14)上发现的恶性浆细胞基因组特征,del(17p),和t(14;16)-评估估计的无进展生存期和总生存期。诊断时,28%的患者被归类为修订的国际分期I期多发性骨髓瘤,这些患者的5年生存率中位数为82%.在所有多发性骨髓瘤患者中,标准一线(诱导)疗法由可注射蛋白酶体抑制剂的组合组成(即,硼替佐米),口服免疫调节剂(即,来那度胺),和地塞米松,并与41个月的中位无进展生存期相关,与8.5个月无治疗的历史报道相比。这种诱导疗法结合自体造血干细胞移植,然后维持来那度胺是符合条件的患者的标准护理。
结论:美国每年约有34920人和全球155688人被诊断为多发性骨髓瘤。注射蛋白酶体抑制剂诱导治疗,口服免疫调节剂和地塞米松,然后用自体造血干细胞移植治疗,来那度胺维持治疗被认为是符合条件的患者的标准治疗。
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