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达雷妥尤单抗治疗累及肾脏的轻链沉积病可预防肾功能进展:1例随访3年的病例报告及文献复习

Daratumumab treatment for kidney-involved light chain deposition disease prevents renal function progression: a case report with 3 years of follow-up and review of the literature.

作者信息

Chen Xueying, Sun Jie, Shen Pingyan, Chen Zijin, Zhang Wen

机构信息

Department of Nephrology, School of Medicine, Ruijin Hospital Shanghai Jiao Tong University, Wuxi, Jiangsu, China.

Department of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

出版信息

Front Oncol. 2025 Jan 30;15:1466323. doi: 10.3389/fonc.2025.1466323. eCollection 2025.

Abstract

Light chain deposition disease (LCDD) is a clonal plasma cell disorder characterized by the deposition of nonamyloid monoclonal light chains in multiple organs. It can affect various systems throughout the body, mainly the kidneys. Symptoms may include renal insufficiency, proteinuria, hematuria, and others. Due to the lack of effective treatment, LCDD patients with kidney involvement often progress to chronic kidney failure, ultimately requiring renal replacement therapy. Daratumumab, an anti-CD38 monoclonal antibody, is primarily used for the treatment of relapsed and refractory multiple myeloma. Recent studies have shown that daratumumab also has an encouraging effect on light-chain amyloidosis. Here, we report the case of an LCDD (κ chain) patient with proteinuria, renal insufficiency, and anemia who was followed up for 3 years, during which he received daratumumab treatment. After the daratumumab treatment, the hematologic response continued progressing to a complete response without any adverse effects and continuous renal function improvement at a low serum free light chain (sFLC) level. This case shows that daratumumab is effective at treating LCDD. For LCDD patients with kidney involvement, frequent monitoring and active control of free light chain levels are necessary, as reaching the lowest sFLC of < 20 mg/L may help to improve kidney function.

摘要

轻链沉积病(LCDD)是一种克隆性浆细胞疾病,其特征是在多个器官中沉积非淀粉样单克隆轻链。它可影响全身各个系统,主要是肾脏。症状可能包括肾功能不全、蛋白尿、血尿等。由于缺乏有效的治疗方法,累及肾脏的LCDD患者常进展为慢性肾衰竭,最终需要肾脏替代治疗。达雷妥尤单抗是一种抗CD38单克隆抗体,主要用于治疗复发难治性多发性骨髓瘤。最近的研究表明,达雷妥尤单抗对轻链淀粉样变性也有令人鼓舞的疗效。在此,我们报告1例LCDD(κ链)患者,该患者有蛋白尿、肾功能不全和贫血,随访3年,期间接受达雷妥尤单抗治疗。达雷妥尤单抗治疗后,血液学反应持续进展至完全缓解,无任何不良反应,且在低血清游离轻链(sFLC)水平时肾功能持续改善。该病例表明达雷妥尤单抗治疗LCDD有效。对于累及肾脏的LCDD患者,需要频繁监测并积极控制游离轻链水平,因为达到最低sFLC < 20 mg/L可能有助于改善肾功能。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d9c/11821651/fce684f7c621/fonc-15-1466323-g001.jpg

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