多发性骨髓瘤伴肾脏病变患者的临床病理特征和预后  

Clinicopathologic characteristics and outcome in patients with multiple myeloma and renal diseases

作　　者：梁丹丹[1] 黄湘华[1] 任贵生[1] 陈文萃[1] 李晓梅[1] 梁少姗[1] 徐峰[1] 刘志红[1] 曾彩虹[1] 

机构地区：[1]南京军区南京总医院肾脏科国家肾脏疾病临床医学研究中心全军肾脏病研究所,南京210016

出　　处：《肾脏病与透析肾移植杂志》2016年第5期401-408,共8页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家科技支撑计划课题(2013BAI09B04;2015BAI12B05);江苏省临床医学科技专项(BL2012007)

摘　　要：目的:分析多发性骨髓瘤(MM)伴肾脏病变患者的肾脏病理类型、临床特征和预后。方法:收集112例临床诊断MM伴肾脏病变并行肾活检的患者的临床、病理及随访资料。分析肾脏病理类型谱及各病理类型的临床和预后差异。结果:MM伴肾脏病变患者以男性多见,46.4%表现肾病范围蛋白尿,9.8%为肾病综合征,镜下血尿和肾功能损害的比例分别占25.9%和68.8%。依据肾活检病理,肾脏病变分MM相关(75.9%)、MM不相关肾脏病变(18.8%)、两者合并存在(1.8%)及肾脏病理基本正常(3.5%)。MM相关肾脏病变以管型肾病(MCN)、淀粉样变性和单克隆免疫球蛋白沉积病(MIDD)最常见。MCN患者行肾活检时血清肌酐水平最高,肾脏病理示肾小管间质急性病变最重。淀粉样变性患者发病时年龄更大,临床多表现肾病综合征,血中升高的轻链以λ为主。MIDD患者临床肾功能不全及血尿发生率高,血中升高的轻链以κ为主,肾脏病理示肾小管萎缩/间质纤维化慢性病变最重。MM相关肾脏病变患者预后显著差于MM不相关肾脏病变患者。对于前者,AMY组肾存活时间优于MCN和MIDD组,但人中位生存时间无统计学差异。结论:MM伴肾脏病变患者的临床及病理特点各有所不同,预后亦存在差异,肾活检有助于明确肾脏病理类型和程度、判断预后。Objective： To investigate clinicopathological features and prognosis in the patients with multiple myeloma and renal diseases. Methodology： One hundred twelve patients diagnosed as multiple myeloma with renal diseases were enrolled into this retrospective study. Their clinical and renal pathological findings were reviewed, and kidney as well as patients＂ survival was also evaluated. Results： They were 78 males and 34 females with male predominance. The clinical features shown that nephrotic proteinuria presented in 46. 4%, and nephritic syndrome in 9.8% of the patients. The percentage of microscopic hematuria and renal insufficiency were 25.9% and 68.8% respectively. According to kidney biopsy findings, renal lesions were divided into muhiple myeloma-related lesions （ 75.9% ） , non-muhiple myeloma-related lesions（ 18. 8%）, combined （1.8%） and almost normal biopsy （ 3.5% ）. Myeloma cast nephropathy （ MCN ） was the commonest multiple myeloma-related renal lesion, followed by monoelonal immunoglobulin deposition disease （MIDD） and amyloidosis. The serum creatinine at renal biopsy was highest and renal pathology also showed prominent acute tubular injury in MCN. Clinical features favoring amyloidosis over MIDD and MCN included older age, absence of kidney failure, presence of nephrotic syndrome and k free chain predominance. The occurrence of hematuria was higher in patients with MIDD than MCN or amyloidosis. Patients with MIDD were more likely to have K free chain in examining serum free light chain and moderate to severe tubular atrophy and interstitial fibrosis in renal histology. The survival of patients with multiple mye＇toma-reiated renal lesions was worse than that of patients with non-multiple myeloma-related renal iesions. In patients with multiple myeloma-related renal lesions, renal survival of patients with AMY was better than that of patients with YICN and MIDD. Conclusion ： The spectrum of renal lesions in patients with multiple myeloma was heterogeneous. Clinical and

关 键 词：多发性骨髓瘤 肾脏病理 预后 

分 类 号：R733.3[医药卫生—肿瘤] R692[医药卫生—临床医学]