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轻链沉积病、骨髓瘤管型肾病以及两者兼具的患者之间的临床和预后差异。

Clinical and prognostic differences among patients with light chain deposition disease, myeloma cast nephropathy and both.

作者信息

Zand Ladan, Nasr Samih H, Gertz Morie A, Dispenzieri Angela, Lacy Martha Q, Buadi Francis K, Kumar Shaji, Kyle Robert A, Fervenza Fernando C, Sethi Sanjeev, Dingli David, Rajkumar S Vincent, Kapoor Prashant, McCurdy Arleigh, Leung Nelson

机构信息

a Division of Nephrology and Hypertension , Rochester , MN , USA.

b Department of Laboratory Medicine and Pathology , Rochester , MN , USA.

出版信息

Leuk Lymphoma. 2015;56(12):3357-64. doi: 10.3109/10428194.2015.1040011. Epub 2015 May 12.

Abstract

In some patients with light chain deposition disease (LCDD) there is also evidence of myeloma cast nephropathy (MCN) on renal biopsy. The purpose of this study was to evaluate the renal and survival outcome of patients with concomitant diagnosis of MCN and LCDD to LCDD and MCN alone. Eighty seven patients were identified and divided into LCDD (n=45), MCN (n=29), and LCDD+ MCN (n=13). Patients with LCDD+ MCN had a worse overall survival (OS) compared to patients with LCDD (p=0.03), but similar to patients with MCN (p=0.4). Death-censored renal survival was no different amongst the groups. Presenting with acute renal failure at time of renal biopsy (HR 7.2, p=0.0002) was an independent poor renal prognostic factor while older age (HR 1.06, p=0.0002), presence of osteolytic lesions (HR 4.4, p<0.0001), and requirement for dialysis or creatinine≥5 mg/dL (HR 3.2, p=0.0006) at time of renal biopsy were independent poor prognostic factors for OS.

摘要

在一些轻链沉积病(LCDD)患者的肾活检中也有骨髓瘤管型肾病(MCN)的证据。本研究的目的是评估同时诊断为MCN和LCDD的患者与单独诊断为LCDD和MCN的患者的肾脏及生存结局。共纳入87例患者,分为LCDD组(n = 45)、MCN组(n = 29)和LCDD + MCN组(n = 13)。与LCDD患者相比,LCDD + MCN患者的总生存期(OS)更差(p = 0.03),但与MCN患者相似(p = 0.4)。各组间死亡校正后的肾脏生存期无差异。肾活检时出现急性肾衰竭(HR 7.2,p = 0.0002)是肾脏预后不良的独立危险因素,而年龄较大(HR 1.06,p = 0.0002)、存在溶骨性病变(HR 4.4,p < 0.0001)以及肾活检时需要透析或肌酐≥5 mg/dL(HR 3.2,p = 0.0006)是OS预后不良的独立危险因素。

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