系统性轻链型淀粉样变性累及肝脏1例

Systemic light chain amyloidosis involving the liver:one case report

目的通过病例报道和文献分析,提高对淀粉样变性的认识和诊治水平。方法回顾2019年2月收治于苏州大学附属第一医院的1例以腹胀纳差为主要表现的系统性轻链(AL)型淀粉样变性的病人的诊疗过程。结果病人因“腹胀纳差”以“肾病综合征”初治,效果不佳,最终行组织学检查明确为淀粉样变性,有多系统累及,经化疗后好转出院。结论淀粉样变性起病形式及临床表现多样,易误诊、漏诊,其中肝脏淀粉样变发病率低,临床表现不典型,缺乏特异性检查指标及影像学表现,诊断困难,诊治过程中应注意体格检查,该病预后不佳,确诊后应尽早开始化疗。

Objective To improve the diagnosis and treatment of amyloidosis through case report and literature analysis.Methods The diagnosis and treatment process of a patient with systemic light chain(AL)amyloidosis mainly manifested as abdominal distension in the First Affiliated Hospital of Soochow University in February 2019 were reviewed.Results The patient was initially treated as"nephrotic syndrome"due to"abdominal distension and anorexia",but the treatment was ineffective.Finally,the histological examination confirmed amyloidosis with multiple systemic involvement.After chemotherapy,the patient was better and discharged from hospital.Conclusions The onset forms and clinical manifestations of amyloidosis are diverse,and it is easy to be misdiagnosed or missed diagnosed.Among them,the incidence of liver amyloidosis is low,with atypical clinical manifestations,and there is a lack of specific examination indicators and imaging findings,so the diagnosis depends on biopsy.The diagnosis of hepatic amyloidosis is difficult,and attention should be paid to physical examination during the diagnosis and treatment.The prognosis of this disease is poor,and chemotherapy should be started as soon as possible after diagnosis.