多发性骨髓瘤伴轻链型淀粉样变性患者的临床研究

发布时间：2018-10-26 14:24

【摘要】：目的:探讨多发性骨髓瘤(multiple myeloma,MM)伴轻链型(AL)淀粉样变性的临床特点、治疗效果及预后。方法:回顾性分析2006年7月-2016年7月10年北京朝阳医院血液科和西院血液科收治的53例新诊断的多发性骨髓瘤伴轻链(AL)型淀粉样变性患者的临床资料,总结其临床表现、治疗及预后情况,对临床特征及疗效进行描述性分析,采用SPSS 22.0软件对相关数据进行统计学分析。结果:多发性骨髓瘤伴AL型淀粉样变性患者,男女比例约为2.1:1(36/17),平均年龄59.9±10.1岁。MM分型为λ轻链型15例(28.3%),Ig G-λ型11例(20.8%),k轻链型10例(18.9%),Ig G-k型9例(17.0%),Ig A-λ型5例(9.43%),Ig D-λ型2例(3.8%),Ig A-k型1例(1.9%)。中位总生存时间OS为12(1-102)个月。累及器官为肾38例(71.70%),心脏33例(62.26%),皮肤17例(32.1%),软组织13例(24.5%),消化道12例(22.6%),肝9例(17.0%),神经系统9例(17.0%),肺1例。中位总生存时间OS为12(1-102)个月。ISSⅡ期患者OS为34(4-81)个月,高于ISSⅢ期患者OS为8(1-102)个月(P0.05)。梅奥分期Ⅰ期患者的OS 38(10-81)个月,高于梅奥分期Ⅱ期患者的OS 8(1-102)个月,高于梅奥分期Ⅲ期的患者OS 1(1-2)个月(P0.05)。1个器官累及患者的OS为37(8-102)个月,高于2个器官累及者的OS为12(1-75)个月,高于3个或以上器官累及的患者OS为5.5(1-38)个月(P0.05)。有心脏累及的患者OS为6(1-36)个月,低于其他患者的中位OS 40(8-102)个月,心脏累及的患者中出现低血压的者的OS为3(1-8)个月,低于无低血压的患者OS 8.5(1-36)个月(P0.05)。此外,出现高危的遗传学改变者OS为8(2-15)个月,显著低于非高危者的OS 26.5(1-102)个月(P0.05)。硼替佐米基础方案有效率高于非硼替佐米基础方案(63.6%vs28.6%)(P0.05),但OS差别无统计学意义。结论:多发性骨髓瘤伴淀粉样变性在临床上少见,常见累及部位为肾脏,其次为心脏。MM患者伴AL型淀粉样变时,OS明显缩短。随ISS分期和梅奥分期的增加,OS逐渐缩短。而且,累及器官越多预后越差,累及心脏者预后最差,心脏累及者出现低血压者预后更差。此外,出现高危的遗传学改变者预后变差。硼替佐米治疗有效率较高,但尚未能转化为生存优势。
[Abstract]:Objective: to investigate the clinical features, therapeutic effect and prognosis of multiple myeloma (multiple myeloma,MM) with light chain (AL) amyloidosis. Methods: the clinical data of 53 newly diagnosed patients with multiple myeloma with light chain (AL) type amyloidosis were analyzed retrospectively from July 2006 to July 2016 in Department of Hematology and Hematology Department of Beijing Chaoyang Hospital. Treatment and prognosis, clinical characteristics and efficacy of descriptive analysis, SPSS 22. 0 software for statistical analysis of relevant data. Results: the male / female ratio of multiple myeloma with AL type amyloidosis was about 2.1: 1 (36 / 17), with an average age of 59.9 卤10.1 years. 15 cases (28.3%) were classified as 位 light chain type. There were 11 cases of Ig G- 位 type (20.8%), k light chain type 10 cases (18.9%), Ig G-k type 9 cases) (17.0%), Ig A- 位 type 5 cases, 9.43%), Ig D- 位 type 2 cases (3.8%). One case of Ig A-k type (1.9%). The median total survival time (OS) was 12 (1-102) months. 38 cases (71.70%) were involved in kidney, 33 cases (62.26%) in heart, 17 cases (32.1%) in skin, 13 cases (24.5%) in soft tissue, 12 cases (22.6%) in digestive tract, 9 cases (17.0%) in liver. There were 9 cases of nervous system (17.0%) and 1 case of lung. The median total survival time (OS) was 12 (1-102) months, OS in ISS 鈪，

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