达雷妥尤单抗治疗难治性有肾脏意义的单克隆免疫球蛋白病的疗效和安全性  

Efficacy and safety of daratumumab in treatment of refractory monoclonal gammopathy of renal significance

作　　者：任贵生 郭锦洲 陈文萃 赵亮 徐维玮 张洋洋 黄湘华 REN Guisheng;GUO Jinzhou;CHEN Wencui;ZHAO Liang;XU Weiwei;ZHANG Yangyang;HUANG Xianghua(National Clinical Research Center for Kidney Diseases,Jinling Hospital,Nanjing 210016,China)

机构地区：[1]东部战区总医院国家肾脏疾病临床医学研究中心,南京210016

出　　处：《肾脏病与透析肾移植杂志》2023年第5期401-405,共5页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家自然科学基金面上项目(82270767)。

摘　　要：目的:观察达雷妥尤单抗治疗复发难治性有肾脏意义的单克隆免疫球蛋白病(MGRS)的疗效和安全性。方法:收集2021年9月至2023年1月在国家肾脏疾病临床医学研究中心接受达雷妥尤单抗治疗的复发难治性MGRS患者的临床资料,分析其血液学缓解、肾脏缓解情况和不良反应。结果:共纳入5例复发难治性MGRS患者,年龄为51(50,57)岁,病程58(23,59)月。轻链沉积病(LCDD)2例,伴有单克隆免疫球蛋白沉积的增生性肾小球肾炎(PGNMID)2例,单克隆免疫球蛋白相关的C3肾小球病1例。在接受达雷妥尤单抗治疗后,2例基线血差值轻链>50 mg/L的患者中有1例实现血液学完全缓解,1例达到部分缓解,而3例血差值轻链20~50 mg/L的患者中有2例降至<10 mg/L。中位随访10(9,12)月,5例患者均存活,2例获得肾脏缓解。输注相关的不良反应包括咳嗽、寒战、咽喉不适,均为1级,无患者因输注反应中断治疗。此外,1例患者出现非感染性的低热,1例出现轻度的中性粒细胞减少,均可迅速恢复。结论:对于复发难治性MGRS患者,达雷妥尤单抗的血液学缓解率高,安全性好,部分患者可获得较好的肾脏反应。Objective:To observe the clinical efficacy and safety of daratumumab in treatment of relapse/refractory monoclonal gammopathy of renal significance(MGRS).Methodology:Patients suffered relapse/refractory MGRS and received the treatment of daratumumab from Septmeber 2021 to January 2023 were enrolled in the study.The clinical data was collected to evaluate hematological and renal responses,adverse reactions were analyzed.Results:Five patients were enrolled,with a median age of 51(50,57)years and a median disease duration of 58(23,59)months.Two patients were diagnosed light chain deposition disease(LCDD),two patients were diagnosed proliferative glomerulonephritis with monoclonal immunoglobulin deposition(PGNMID)and the other one was monoclonal immunoglobulin-related C3 glomerulopathy.After receiving of daratumumab,one of the two patients with difference free light chain50 mg/L achieved hematological complete response and the other one achieved partial response,while two of the three patients with difference free light chain between 20~50 mg/L decreased to 10 mg/L.After a follow-up of 10(9,12)months,all five patients were alive,and two achieved renal response.Infusion-related adverse reactions included cough,chills and throat discomfort,and all were classified to grade 1.In addition,one patient had low degree fever unrelated to infection,and mild neutropenia occured in one patient.Conclusion:Daratumumab is effective and safe in treatment of relapse/refractory MGRS,the hematological response rate was high,and renal response was observed in some patients.

关 键 词：达雷妥尤单抗 有肾脏意义的单克隆免疫球蛋白病 疗效 安全性 

分 类 号：R692[医药卫生—泌尿科学]