罕见系统性轻链型淀粉样变性累及多系统1例  

Rare Systemic Light-Chain Amyloidosis Involving Multi System Injury:A Case Report

作　　者：徐慧霞 阿茹娜 徐长荣 王颖 云莎 XU Hui-xia;ARu-na;XU Chang-rong;WANG Ying;YUN Sha(Department of Blood specialty,Inner Mongolia People’s Hospital,Hohhot Inner Mongolia,010017,China)

机构地区：[1]内蒙古自治区人民医院血液科,内蒙古呼和浩特010017

出　　处：《中华养生保健》2024年第19期13-17,共5页CHINESE HEALTH CARE

基　　金：内蒙古医科大学联合项目(YKD2022LH073)。

摘　　要：目的通过对就诊于内蒙古自治区人民医院的1例罕见系统性轻链型淀粉样变性累及多系统病例的诊断、治疗过程进行病例报道和相关分析,提高对该疾病的认知和诊治水平。方法收集2023年4月就诊于内蒙古自治区人民医院的1例以血尿、水肿为首发症状,后经系统诊治确诊为系统性轻链型淀粉样变性的诊疗过程资料。结果患者的初步诊断为“肾病综合征”,相关治疗效果不佳,经多学科会诊,最终完善相关检查,确诊为淀粉样变性并多系统累及,经对症支持治疗(化疗及肾脏替代治疗)后好转出院。结论淀粉样变性临床上诊断困难,容易误诊、漏诊,一经诊断应尽快进行相关治疗,肾脏受累者联合透析有望恢复肾脏功能,希望通过病例报道,提高对该疾病的认知,减少漏诊、误诊。Objective To report and analyze relevant literature on a rare case of systemic light chain amyloidosis involving multiple systems treated in Inner Mongolia People’s Hospital,in order to improve our understanding and diagnosis of diseases.Methods Collect the diagnosis and treatment process of a rare systemic light chain amyloidosis patient with hematuria and edema as the initial symptoms who visited Inner Mongolia Autonomous Region People''s Hospital in April 2023.Results The patient was initially diagnosed with nephrotic syndrome,but the treatment effect was not satisfactory.Finally,multi-disciplinary consultation,a bone marrow biopsy was performed and Congo red staining was performed to confirm the diagnosis of amyloidosis,involving multiple systems.After chemotherapy and renal replacement therapy,the patient improved and was discharged.Conclusion The onset and clinical manifestations of amyloidosis are diverse,diffi cult to diagnose,and prone to misdiagnosis and missed diagnosis.Once diagnosed,chemotherapy should be administered as soon as possible.For kidney aff ected patients,combined dialysis is expected to restore kidney function.It is hoped that through case reports,we can improve our understanding of the disease and reduce missed diagnosis and misdiagnosis.

关 键 词：系统性轻链型淀粉样变性 多系统损害 血尿 

分 类 号：R559[医药卫生—血液循环系统疾病]