伴有髓外病变的多发性骨髓瘤65例临床诊治分析  

Clinical features analysis of 65 cases of multiple myeloma with extramedullary disease

作　　者：沈继春 张宇辰 李杨 邓琦[4] 徐朋朋 Shen Jichun;Zhang Yuchen;Li Yang;Deng Qi;Xu Pengpeng(Department of Endocrinology and Hematology of the Characteristic Medical Center,Tianjin 300162,China;Department of General Practice of the Characteristic Medical Center,Tianjin 300162,China;Orthopedics Department of the Characteristic Medical Center,Tianjin 300162,China;Department of Hematology,Tianjing First Central Hospital of Nankai University,Tianjin 300110,China;oncology department of the Characteristic Medical Center,Tianjin 300162,China)

机构地区：[1]武警部队特色医学中心内分泌与血液科,天津300162 [2]武警部队特色医学中心全科医学科,天津300162 [3]武警部队特色医学中心骨科,天津300162 [4]天津市第一中心医院血液科,天津300110 [5]武警部队特色医学中心肿瘤科,天津300162

出　　处：《中国综合临床》2024年第3期206-211,共6页Clinical Medicine of China

基　　金：北京希思科临床肿瘤学研究基金会科研基金(Y-NCJH202201-0027)。

摘　　要：目的研究多发性骨髓瘤(multiple myeloma,MM)合并髓外病变的临床特点、治疗方案及预后。方法选取2014年1月至2020年5月在武警部队特色医学中心及天津市第一中心医院收治的65例患者的临床资料进行回顾性分析,年龄范围22~79岁,中位年龄为56(22,79)岁,女性与男性比例为27/38。分别接受化学治疗、放射治疗或自体造血干细胞移植(autologous hematopoietic stem cell transplantation,AHSCT)治疗。65例患者均采用荧光原位杂交(fluorescence in situ hybridization,FISH)技术检测t(4;14)、t(14;16)、1q21、del(17p)。非正态分布的计量资料以M(Q_(1),Q_(3))表示,采用Kaplan-Meier法构建生存曲线。结果所有患者多处骨质发生溶骨性病变,β2微球蛋白和乳酸脱氢酶均升高。最常见染色体异常率:t(4;14)为10.8%,t(14;16)为16.9%,1q21为18.5%,del(17p)为21.5%。7.7%的患者1q21和del(17p)共存。65例患者接受V-DRPACE方案治疗后,有效率为89.2%(58/65)。32例部分缓解的患者接受了放射治疗,放射治疗完全缓解率为18.8%(6/32)。20例符合移植条件的患者行AHSCT治疗,13例患者实现完全缓解。随访结束时,65例患者的中位总生存期为44.8个月,无进展生存期为11个月。结论MM合并髓外病变通常累及多部位,易出现多发溶骨性病变,β2微球蛋白和乳酸脱氢酶升高。此类患者细胞遗传学异常率明显增高。V-DRPACE方案对合并髓外病变的MM患者治疗有效且可以从AHSCT中获益。Objective To study the clinical features,treatment and prognosis of multiple myeloma(MM)complicated with extramedullary disease(EMD).Methods A total of 65 patiens admitted to the Characteristic Medical Center and Tianjing First Central Hospital from January 2014 to May 2020 were analyzed retrospectively as the study subjects.The age ranged from 22 to 79 years,with a median age of 56(22,79)years,and the ratio of female to male was 27/38.They were respectively treated by chemotherapy,radiotherapy or autologous hematopoietic stem cell transplantation(AHSCT).Fluorescence in situ hybridization(FISH)was used in all 65 patients to detect t(4;14),t(14;16),1q21,del(17p).The measurement data of non-normal distribution were expressed in M(Q1,Q3),and the survival curve was constructed by Kaplan-Meier method.Result The increase of β2-microglobulin and lactate dehydrogenase,multiple osteolytic lesions were found in all patients.The most common rate of chromosomal abnormalities were10.8%for t(4;14),16.9% for t(14;16),18.5% for 1q21,21.5% for del(17p).Both 1q21 and del(17p)were co-existed in 7.7% of patients.The effective rate of 65 patients treated with V-DRPACE regimen was 89.2%(58/65).Thirty-two patients who were in partial remission were treated with radiotherapy.The complete remission rate of radiotherapy was 18.8% (6/32).Twenty patients eligible for transplantation underwent AHSCT and 13 patients achieved complete remission.At the end of follow-up,the median overall survival(OS)of 65 patients was 44.8 months,and progression-free survival(PFS)was11 months.Conclusion MM with EMD usually involve multiple sites,prone to multiple osteolytic lesions,increased β2-microglobulin and lactate dehydrogenase.The rate of cytogenetic abnormalities in such patients is significantly increased.The V-DRPACE regimen is effective in treating MM patients with extramedullary lesions and can benefit from AHSCT.

关 键 词：多发性骨髓瘤 髓外疾病 自体造血干细胞移植 

分 类 号：R733.3[医药卫生—肿瘤]