1例系统性轻链型淀粉样变性继发凝血因子Ⅹ、Ⅶ、Ⅸ缺乏导致致命性消化道出血的报道  

A case study of systemic light⁃chain amyloidosis with secondary deficiency in coagulation factors Ⅹ,Ⅶ,and Ⅸ resulting in fatal gastrointestinal hemorrhage

作　　者：张小梅[1] 汪春晓 袁丽娟 陈为志[1] 李镜[1] Zhang Xiaomei;Wang Chunxiao;Yuan Lijuan;Chen Weizhi;Li Jing(Department of Hematology,People’s Hospital of Rizhao,Rizhao 276800,China)

机构地区：[1]日照市人民医院血液科,日照276800

出　　处：《血栓与止血学》2025年第1期31-35,共5页Chinese Journal of Thrombosis and Hemostasis

摘　　要：系统性轻链(systemic light⁃chain,AL)型淀粉样变性是一种罕见的多脏器受累的系统性疾病,临床诊断治疗困难。本文报道1例以消瘦、大便次数增多为初期表现的老年AL型淀粉样变性患者,凝血功能示凝血酶原时间(prothrombin time,PT)、部分凝血活酶时间(activated partial thromboplastin time,APTT)延长,凝血因子活性示凝血因子Ⅹ、Ⅶ、Ⅸ缺乏,消化内镜下以充血、出血为主要表现,骨髓及胃体活组织检查刚果红染色阳性,由于凝血因子替代治疗疗效不佳,患者消化道出血反复,短期内临床死亡。旨在通过本病例的诊治经验及对国内外文献复习,引起临床医师对AL型淀粉样变性胃肠道受累、凝血功能异常的关注。Systemic light⁃chain(AL)amyloidosis is a rare systemic disease with multiple organ involvement,which is difficult to diagnose and treat clinically.This article reports a case of an elderly patient with AL amyloidosis who initially experienced weight loss and increased bowel movements.The coagulation function showed prolonged prothrombin time(PT)and activated partial thromboplastin time(APTT),and the activity of coagulation factors showed the deficiency of coagulation factorsⅩ,Ⅶ,andⅨ.The main manifestations under digestive endoscopy were congestion and bleeding,and Congo red staining was positive in bone marrow and gastric body biopsies.Despite coagulation factor replacement therapy,the patient suffered recurrent gastrointestinal bleeding and died shortly after.This case highlights the importance of recognizing gastrointestinal and coagulation issues in AL amyloidosis by sharing our experience and reviewing relevant literature.

关 键 词：系统性轻链型淀粉样变性 获得性凝血因子缺乏 消化道出血 凝血因子Ⅹ、Ⅶ、Ⅸ 

分 类 号：R554[医药卫生—血液循环系统疾病]