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中华肾病研究电子杂志 ›› 2024, Vol. 13 ›› Issue (06) : 352 -357. doi: 10.3877/cma.j.issn.2095-3216.2024.06.009

所属专题: 经典病例

病例报告

达雷妥尤单抗治疗复发及难治性轻链沉积病:1 例报告及文献复习
鲜华明1, 谢席胜1,2,,3   
  1. 1.637000 川北医学院附属南充市中心医院肾内科
    2.637000 南充市慢性肾脏病基础与临床研究重点实验室
    3.637000 南充市临床医学研究中心
  • 收稿日期:2023-12-25 出版日期:2024-12-28
  • 通信作者: 谢席胜
  • 基金资助:
    四川省中医药管理局科研专项基金(2020JC0079)四川省科技厅科研专项基金(2021YFS0259)

Daratumumab treatment for relapsed and refractory light chain deposition disease:a case report with literature review

Huaming Xian, Xisheng Xie()   

  • Received:2023-12-25 Published:2024-12-28
  • Corresponding author: Xisheng Xie
引用本文:

鲜华明, 谢席胜. 达雷妥尤单抗治疗复发及难治性轻链沉积病:1 例报告及文献复习[J/OL]. 中华肾病研究电子杂志, 2024, 13(06): 352-357.

Huaming Xian, Xisheng Xie. Daratumumab treatment for relapsed and refractory light chain deposition disease:a case report with literature review[J/OL]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2024, 13(06): 352-357.

图1 患者肾脏组织病理 注:A:HE 染色×100;B:PAS 染色×400;C:PASM 染色×100;D:Masson 染色×400
图2 患者肾脏组织免疫荧光 注:A:ALB( -)(免疫荧光×200);B: 肾小球、肾小管基底膜及小动脉κ( +)(免疫荧光×200);C:肾小球、肾小管基底膜及小动脉λ( -)(免疫荧光×200)
图3 患者肾脏组织电镜表现 注:A:电镜×6 000;B:电镜×3 000;毛细血管内皮细胞明显空泡变性,肾小球基底膜节段性增厚,足突广泛融合,系膜细胞和基质明显增生,结节形成;系膜区、肾小球基底膜内侧、肾小管基底膜外侧可见较多颗粒状无定形的电子致密物
图4 患者24 h 尿蛋白定量变化
图5 患者肾功能变化
表1 轻链沉积病各类治疗方案比较
研究者(时间) 研究类型 患者人数(例)[性别,中位年龄(岁)] 受累器官 治疗方案 疾病转归及疗效
Petrakis等(2010)[13] 病例报道 1(女,49) 肾脏 HDM+ASCT 首次报道:ASCT后肾LCDD消退
Abdallah等(2020)[14] 回顾性研究 11(未知) 心脏 不同剂量美法仑+ASCT(9例)或单纯化疗(2例) ASCT患者未达中位总生存期、仅化疗患者中位总生存期为2.5个月(P=0.014);35% ASCT患者经历严重不良事件,65% ASCT患者耐受良好
González-L等(2021)[15] 病例报道 1(男,28) HDM+ASCT ASCT9月后肺功能稳定游离轻链正常
González-López等(2011)[16] 病例报道 1(女,63) 肾脏 初始:BD;随后:HDM+ASCT 治疗后近五年持续达严格意义的完全缓解状态
Adamu等(2016)[17] 病例报道 1(男,38) 肾脏 初始:CyBorD;随后:TCD 蛋白尿<1g/d且未测得游离轻链达完全缓解
Dutta等(2020)[18] 病例报道 1(女,63) 肾脏 诱导:CyBorD;维持:BD 血清肌酐1.4 mg/dl,κ/λ比值2.55
Milani等(2020)[19] 回顾性研究 8(男6/女2,61.5) 肾脏 病情进展后:DARA 4例达非常好的部分缓解;7例患者达部分缓解;6例eGFR持续稳定;2例进展为终末期肾病
Tsushima等(2021)[20] 病例报告 1(女,66) 肝、肾 DARA-CyBorD 血清肌酐降至3.19 mg/dl;κ游离轻链降至20.2 mg/L;κ/λ比值恢复正常达完全缓解
Kastritis等(2021)[21] 回顾性研究 6 (男4/ 女2,67. 5) 肾脏 初始:CyBorD;短程巩固:DARA 1例患者达完全缓解,1例达非常好的部分缓解;DARA巩固治疗可改善血液反应深度
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