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目的:评估多发性骨髓瘤(multiple myeloma,MM)合并肾淀粉样变性的临床特征、疗效及预后。方法:回顾性分析我院2011年11月-2015年07月期间新发的22例MM合并肾淀粉样变性患者的临床资料。结果:22例患者ISS分期Ⅱ期占77.2%(17/22),Ⅲ期为22.8%(5/22)。肾功能损害占36.4%(8/22),贫血占40.9%(9/22),血清白蛋白<35 g/L占86.4%(19/22),尿蛋白阳性100%(22/22)。22例中完全缓解(CR)13.6%(3/22),非常好的部分缓解(VGPR)4.5%(1/22),部分缓解(PR)22.8%(5/22),疾病稳定(SD)45.5%(10/22),疾病进展(PD)13.6%(3/22),其中9例治疗有效患者,肾脏淀粉样变性有3例改善,4例稳定,2例进展。可随访17例患者中,7例死亡,10例存活,平均随访11(1-37)个月,中位总体生存19(95%CI 9.2-28.8)个月。结论:MM合并肾淀粉样变性临床上少见、难治、预后差。MM出现大量蛋白尿特别是合并肾病综合征时,无论是否有肾功能损害,均需考虑合并有肾淀粉样变性。以硼替佐米为基础的化疗可能提高患者疗效。
Objective: To evaluate the clinical features, efficacy and prognosis of multiple myeloma (MM) combined with renal amyloidosis. Methods: The clinical data of 22 newly diagnosed MM patients with renal amyloidosis in our hospital from November 2011 to July 2015 were retrospectively analyzed. Results: Among the 22 patients, 77.2% (17/22) were ISS stage Ⅱ and 22.8% (5/22) in stage Ⅲ. Renal dysfunction accounted for 36.4% (8/22), anemia accounted for 40.9% (9/22), serum albumin <35 g / L accounted for 86.4% (19/22), urine protein positive 100% (22/22). Among the 22 patients, CR was 13.6% (3/22), VGPR 4.5% (1/22), partial response (22.8%), stable disease (SD) 45.5% (10/22), and 13.6% (3/22) of disease progression (PD). Of the 9 patients who were treated effectively, 3 were improved amyloidosis, 4 were stable, and 2 were progressed. Of the 17 patients who were followed, 7 were fatal and 10 were alive with an average follow-up of 11 (1-37) months, with a median overall survival of 19 (95% CI 9.2-28.8) months. Conclusion: MM combined with renal amyloidosis is clinically rare, refractory, with poor prognosis. MM, a large number of proteinuria, especially with nephrotic syndrome, whether or not there is renal damage, should be considered with renal amyloidosis. Chemotherapy based on bortezomib may improve patient outcomes.