摘要
目的分析轻链沉积病(LCDD)的临床特点及预后情况。方法回顾性分析西京医院2015-2020年收治的经肾活检证实的LCDD患者的其临床资料、肾脏病理类型、治疗及预后情况。结果共纳入13例患者,男女比例为3.3∶1.0,年龄53.77(36.00~68.00)岁。临床表现为肾病综合征范围蛋白尿(46.1%)、肾功能不全(76.9%)、高血压(84.6%)。30.8%合并多发性骨髓瘤。血清游离轻链检测显示,免疫固定电泳阳性率分别为84.6%。光镜下可见弥漫性肾小管基底膜增厚(100.0%)、结节性肾小球硬化(84.6%),荧光下肾小管基底膜可见线性轻链限制性染色(κ染色阳性10例,λ染色阳性3例),电镜下均可见肾小管基底膜外和(或)肾小球基底膜内可见粉末状电子致密物沉积。13例患者,除1例起病时已透析外,8例接受了以硼替佐米为基础的治疗方案,4例给予其他治疗。平均随访(27.3±17.76)月,中位肾脏存活率为5.5年;1年、3年的肾存活率分别为67%、54%,以硼替佐米为基础的治疗,与其他治疗相比,1年肾脏存活率明显升高(87%vs.25%,P=0.04)。结论基于硼替佐米的方案治疗LCDD可获得持续的血液学反应,明显改善肾脏存活率。
Objective To analyze the clinical characteristics and prognostic situation of light chain deposition disease(LCDD).Methods The clinical data,renal pathology type,treatment and prognosis situation in the patients with LCDD verified by renal biopsy in Xijing Hospital during 2015-2020 were retrospectively analyzed.Results Thirteen patients were included,the ratio of male to female was 3.3∶1.0,with an average age of 53.77(36.00-68.00)years old.The clinical manifestations were nephrotic syndrome range proteinuria(46.1%),renal insufficiency(76.9%)and hypertension(84.6%).30.8%of patients were complicated with multiple myeloma.The serum free light chain detection showed that the positive rate of immunofixation electrophoresis was 84.6%respectively.The microscope showed the diffuse thickening of renal tubular(100.0%)and nodular glomerulosclerosis(84.6%).The linear light chain restricted staining in renal tubular basal membrane could be seen under fluorescence(κpositive staining in 10 cases,λpositive staining 3 in 3 cases).Electron microscopy showed the deposition of powdered electron density outside the renal tubule basement membrane and/or inside the glomerular basement membrane.Among 13 cases,except 1 case conducted the dialysis in disease onset,8 cases received the treatment scheme with bortezomib as base,and 4 cases were given other treatment.The average follow up was(27.3±17.76)months.The median renal survival rate was 5.5 years.The 1-year and 3-year renal survival rates were 67.5%and 54.0%respectively.Bortezomib served as the basic treatment,compared with other treatment,the 1-year renal survival rate was significantly increased(87%vs.25%,P=0.04).Conclusion The bortezomib-based scheme for treating LCDD can achieve the sustained hematological response and significantly improve the renal survival rate.
作者
田秀娟
何娟
柳红昌
TIAN Xiujuan;HE Juan;LIU Hongchang(Department of Nephrology,Xijing Hospital,Xi′an,Shaanxi 710032,China;Department of Anesthesiology,Xijing Hospital,Xi′an,Shaanxi 710032,China)
出处
《重庆医学》
CAS
2021年第14期2423-2428,共6页
Chongqing medicine
关键词
轻链沉积症
硼替佐米
肾脏
存活率
light chain deposition disease
bortezomib
kidney
survival rate
