达雷妥尤治疗1例难治性重链沉积病  

Refractory heavy chain deposition disease treated with daratumumab

作　　者：李银芳 蔡惠丽 姚碧晴 孙博文 邹雅倩 史明君 杨林[1] LI Yinfang;CAI Huili;YAO Biqing;SUN Bowen;ZOU Yaqian;SHI Mingjun;YANG Lin(Department of Nephrology,The First College of Clinical Medical Science,China Three Gorges University&Yichang Central People's Hospital,Yichang 443003,China;Department of Hematology,The First College of Clinical Medical Science,China Three Gorges University&Yichang Central People's Hospital,Yichang 443003,China)

机构地区：[1]三峡大学第一临床医学院,宜昌市中心人民医院肾病内科,宜昌443003 [2]三峡大学第一临床医学院宜昌市中心人民医院血液内科

出　　处：《肾脏病与透析肾移植杂志》2024年第3期295-300,共6页Chinese Journal of Nephrology,Dialysis & Transplantation

摘　　要：中年女性患者,因“间断双下肢伴颜面部水肿7月余,恶心呕吐2 d”就诊。该患者临床症状无特异性,肾脏损伤表现为蛋白尿、镜下血尿、肾功能异常,低补体血症。组织学改变为肾小球结节性硬化伴系膜增生性肾小球肾炎,免疫荧光见IgG1、补体C3大量在肾小球系膜区团块状沉积,并沿着肾小球毛细血管袢及肾小管基膜线性沉积,电镜下肾小球部分系膜区可见直径为18~20 nm的细颗粒或细纤维样物质。血清M蛋白质谱分析为重链γ丰度最高,轻链λ与κ相对丰度较低,诊断为重链沉积病伴纤维样结构沉积。予硼替佐米为基础的治疗后患者肾功能好转,血液学部分缓解,但停药后复发,继续予硼替佐米为基础的治疗,病情无缓解,转为以达雷妥尤单抗为基础的治疗方案,达到血液学非常好的部分缓解。A middle-aged female patient presented with“intermittent bilateral lower extremity edema with facial edema for more than 7 months,nausea and vomiting for 2 days”.The clinical symptoms of this case were non-specific.The kidney injury showed proteinuria,microscopic hematuria,abnormal renal function,and hypocomplementeemia.The main pathological features were glomerular nodular sclerosis with mesangial proliferative glomerulonephritis.Immunofluorescence showed massive deposition of IgG1 and complement C3 in the mesangial region,and linear deposition along the glomerular vascular loops and the basement membrane of the renal tubules.Fine particles or fine fibril with a diameter of 18~20 nm could be seen in the mesangial region of the glomerulus.Serum M protein spectrum analysis showed the highest abundance of heavy chainγ,low relative abundance of light chainλandκ.Heavy chain deposition disease was diagnosed with fibrous structure deposition.After bortezomib based treatment,renal function improved and hematologic partial remission was achieved.However,the disease recurred after drug withdrawal,continued bortezomib based treatment,and the disease did not remission,and then changed to daratumumab based treatment,and hematologic partial remission was achieved.

关 键 词：重链沉积病 浆细胞病 达雷妥尤单抗 

分 类 号：R692[医药卫生—泌尿科学]