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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 analyze the renal pathological types, clinical features and prognosis in patients with multiple myeloma (MM) and nephropathy. Methods: The clinical, pathological and follow-up data of 112 patients with MM diagnosed as renal biopsy with renal disease were collected. Analysis of renal pathological type spectrum and the various pathological types of clinical and prognostic differences. Results: MM patients with renal disease were more common in men, 46.4% showed proteinuria in nephrotic range, 9.8% were nephrotic syndrome, 25.9% and 68.8% in microscopic hematuria and renal dysfunction respectively. According to the pathology of renal biopsy, MM was associated with MM (75.9%), MM with unrelated MM (18.8%), both of them were combined (1.8%) and renal pathology was normal (3.5%). MM-related nephropathy with tubular nephropathy (MCN), amyloidosis and monoclonal immunoglobulin deposition disease (MIDD) is the most common. MCN patients had the highest serum creatinine level during renal biopsy and renal pathology showed the most severe renal interstitial lesions. Patients with amyloidosis at the onset of a larger age, clinical manifestations of nephrotic syndrome, elevated blood light chain to lambda. MIDD patients with clinical renal insufficiency and high incidence of hematuria, elevated blood in the light chain to κ-based, renal pathology showed renal tubular atrophy / interstitial fibrosis chronic lesions the most. The prognosis of MM-related nephropathy patients was significantly worse than that of MM-unrelated kidney disease patients. For the former, the survival time of AMY group was better than that of MCN and MIDD group, but the median survival time was not statistically different. Conclusion: The clinical and pathological features of patients with MM are different and their prognosis is different. The renal biopsy can help clarify the type and degree of renal pathology and determine the prognosis.