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伴有肾损害的副蛋白血症患者的临床病理特征。

Clinicopathological characteristics of patients with paraproteinemia and renal damage.

机构信息

Department of Nephrology, Key Laboratory of Kidney Disease Prevention and Control Technology, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, 310007, China.

出版信息

Eur J Med Res. 2021 Jul 3;26(1):68. doi: 10.1186/s40001-021-00538-2.

Abstract

BACKGROUND

This study aimed to analyze the clinicopathological characteristics of patients with paraproteinemia and renal damage.

METHODS

Ninety-six patients from 2014 to 2018 with paraproteinemia and renal damage were enrolled and the clinical data, renal pathology, treatment and prognosis data were collected.

RESULTS

A total of 96 patients (54 male and 42 female), accounting for 2.7% of all renal biopsies, were enrolled in this study. Among them, 42 were monoclonal gammopathy of renal significance (MGRS), 21 were renal monotypic immunoglobulin alone (renal monoIg), and 19 were monoclonal gammopathy of undetermined significance (MGUS). Individuals with multiple myeloma (MM) accounted for the fewest number of patients (n  =  14). In the MGRS group, the main diseases were amyloidosis (n  =  25) and cryoglobulinemic glomerulonephritis (n  =  7), while in the MM group, the main diseases were cast nephropathy (n  =  9) and light chain deposit disease (n  =  3). In the MGUS group, it was mainly IgA nephropathy (IgAN, n  =  10) and idiopathic membranous nephropathy (n  =  5); while in the renal monoIg group, most of the cases were IgAN (n  =  19). Chemotherapy was mainly administered to patients in the MM group, while immunosuppression therapy was mostly administered to patients in the renal monoIg group. Most patients with renal monoIg exhibited a major response, followed by the patients with MGUS and MGRS, while most of the patients with MM had a partial response but none had a major response. Approximately more than half (57.1%) of the patients with MM progressed to end-stage renal disease (ESRD), followed by MGRS (33.3%); however, the mortality rate was low in both the MGRS and MM groups. The survival analysis reviewed that serum creatinine, hemoglobin levels, and the serum κ/λ ratio were independent risk factors for ESRD in patients with MGRS.

CONCLUSIONS

The clinicopathological changes in patients with MGRS were between those in patients with MM and MGUS. The treatment for MGRS and MM was more intensive, and the overall mortality rate was low. Both MGUS and renal monoIg alone exhibited slighter clinicopathological features than MGRS and MM, and the treatment was focused mostly on primary renal diseases.

摘要

背景

本研究旨在分析伴有肾损害的副蛋白血症患者的临床病理特征。

方法

纳入 2014 年至 2018 年期间 96 例伴有肾损害的副蛋白血症患者,收集其临床资料、肾病理、治疗及预后资料。

结果

共纳入 96 例患者(男 54 例,女 42 例),占同期肾活检的 2.7%。其中,单克隆丙种球蛋白病肾意义(MGRS)42 例,单纯单克隆免疫球蛋白肾损害(肾单 Ig)21 例,意义未明单克隆丙种球蛋白血症(MGUS)19 例。多发性骨髓瘤(MM)患者最少(n  =  14)。MGRS 组中,主要疾病为淀粉样变性(n  =  25)和冷球蛋白血症性肾小球肾炎(n  =  7),MM 组中,主要疾病为铸型肾病(n  =  9)和轻链沉积病(n  =  3)。MGUS 组主要为 IgA 肾病(IgAN,n  =  10)和特发性膜性肾病(n  =  5);而在肾单 Ig 组中,IgAN 最多(n  =  19)。MM 组主要给予化疗,肾单 Ig 组主要给予免疫抑制治疗。肾单 Ig 组大部分患者主要反应,MGUS 和 MGRS 组次之,MM 组大部分患者部分反应,无主要反应。MM 组半数以上(57.1%)患者进展为终末期肾病(ESRD),其次为 MGRS(33.3%);但 MGRS 和 MM 组死亡率均较低。生存分析显示,MGRS 患者的血清肌酐、血红蛋白水平和血清 κ/λ 比值是 ESRD 的独立危险因素。

结论

MGRS 患者的临床病理变化介于 MM 和 MGUS 之间。MGRS 和 MM 的治疗更积极,总体死亡率较低。MGUS 和单纯肾单 Ig 比 MGRS 和 MM 的临床病理特征更轻,治疗主要集中在原发性肾脏疾病上。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd23/8255003/40aa47180632/40001_2021_538_Fig1_HTML.jpg

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