以肝肿大为主要表现的多器官AL型淀粉样变性1例  

A case of amyloid light amyloidosis involving multiorgan with hepatomegaly as the main manifestation

作　　者：陈亚男 刘家龙 李奕璋 张记霞 施先艳[1,2,3] 刘静 Ya-Nan CHEN;Jia-Long LIU;Yi-Zhang LI;Ji-Xia ZHANG;Xian-Yan SHI;Jing LIU(Department of Gastroenterology,Zhongnan Hospital of Wuhan University,Wuhan 430071,China;Hubei Clinical Center of Intestinal and Colorectal Diseases,Wuhan 430071,China;Hubei Key Lab of Intestinal and Colorectal Diseases,Wuhan 430071,China)

机构地区：[1]武汉大学中南医院消化内科,武汉430071 [2]湖北省肠病医学临床研究中心,武汉430071 [3]肠病湖北省重点实验室,武汉430071

出　　处：《医学新知》2023年第4期285-290,共6页New Medicine

基　　金：国家自然科学基金面上项目(82072753)。

摘　　要：免疫球蛋白轻链(amyloid light,AL)型淀粉样变性也称为原发性系统性淀粉样变性,是一种蛋白质构象疾病,是由于不溶性纤维蛋白的异常折叠和沉积在器官或组织的细胞外区域,致使受累脏器功能衰竭的一种临床综合征。由于AL型淀粉样变性发病率低且临床表现复杂,其诊断具有挑战性,多器官累及的病例更加罕见。本文报道了1例多器官累及的AL型淀粉样变性,旨在为相关临床诊疗及预后提供参考。Amyloid light(AL)amyloidosis,also known as primary systemic amyloidosis,is a protein conformation disease,and is a clinical syndrome of functional failure of affected organs due to abnormal folding and deposition of insoluble fibrin in the extracellular region of organs or tissues.The diagnosis of AL-type amyloidosis is challenging because of its low incidence and complex clinical manifestations,and cases of multiple organ involvement are even rarer.We reported a case of AL-type amyloidosis with multiple organ involvement in order to provide refference for related diagnosis,treatment and prognosis.

关 键 词：淀粉样变性 轻链型淀粉样变性 多器官累及 

分 类 号：R597[医药卫生—内科学]