【摘要】：目的:探討多發(fā)性骨髓瘤(multiple myeloma,MM)伴輕鏈型(AL)淀粉樣變性的臨床特點、治療效果及預后。方法:回顧性分析2006年7月-2016年7月10年北京朝陽醫(yī)院血液科和西院血液科收治的53例新診斷的多發(fā)性骨髓瘤伴輕鏈(AL)型淀粉樣變性患者的臨床資料,總結其臨床表現(xiàn)、治療及預后情況,對臨床特征及療效進行描述性分析,采用SPSS 22.0軟件對相關數(shù)據進行統(tǒng)計學分析。結果:多發(fā)性骨髓瘤伴AL型淀粉樣變性患者,男女比例約為2.1:1(36/17),平均年齡59.9±10.1歲。MM分型為λ輕鏈型15例(28.3%),Ig G-λ型11例(20.8%),k輕鏈型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%)。中位總生存時間OS為12(1-102)個月。累及器官為腎38例(71.70%),心臟33例(62.26%),皮膚17例(32.1%),軟組織13例(24.5%),消化道12例(22.6%),肝9例(17.0%),神經系統(tǒng)9例(17.0%),肺1例。中位總生存時間OS為12(1-102)個月。ISSⅡ期患者OS為34(4-81)個月,高于ISSⅢ期患者OS為8(1-102)個月(P0.05)。梅奧分期Ⅰ期患者的OS 38(10-81)個月,高于梅奧分期Ⅱ期患者的OS 8(1-102)個月,高于梅奧分期Ⅲ期的患者OS 1(1-2)個月(P0.05)。1個器官累及患者的OS為37(8-102)個月,高于2個器官累及者的OS為12(1-75)個月,高于3個或以上器官累及的患者OS為5.5(1-38)個月(P0.05)。有心臟累及的患者OS為6(1-36)個月,低于其他患者的中位OS 40(8-102)個月,心臟累及的患者中出現(xiàn)低血壓的者的OS為3(1-8)個月,低于無低血壓的患者OS 8.5(1-36)個月(P0.05)。此外,出現(xiàn)高危的遺傳學改變者OS為8(2-15)個月,顯著低于非高危者的OS 26.5(1-102)個月(P0.05)。硼替佐米基礎方案有效率高于非硼替佐米基礎方案(63.6%vs28.6%)(P0.05),但OS差別無統(tǒng)計學意義。結論:多發(fā)性骨髓瘤伴淀粉樣變性在臨床上少見,常見累及部位為腎臟,其次為心臟。MM患者伴AL型淀粉樣變時,OS明顯縮短。隨ISS分期和梅奧分期的增加,OS逐漸縮短。而且,累及器官越多預后越差,累及心臟者預后最差,心臟累及者出現(xiàn)低血壓者預后更差。此外,出現(xiàn)高危的遺傳學改變者預后變差。硼替佐米治療有效率較高,但尚未能轉化為生存優(yōu)勢。
[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 鹵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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