Hong Seonghun, Chang Young Woon, Byun Jong Kyu, Kim Min Je, Chae Jung Min, Park Sun Hee, Oh Chi Hyuk, Park Yong Koo
Department of Internal Medicine, Kyung Hee University School of Medicine, Seoul, Korea.
Department of Pathology, Kyung Hee University School of Medicine, Seoul, Korea.
Korean J Gastroenterol. 2015 Oct;66(4):227-30. doi: 10.4166/kjg.2015.66.4.227.
A 53-year-old woman was admitted with epigastric discomfort and weakness. Laboratory examination at admission showed mild anemia and proteinuria. Esophagogastroduodenoscopy revealed marked mucosal atrophy, diffuse nodularity and granular appearance with mucosal friability. Biopsy was performed on the antrum and body of the stomach. On the next day, the patient began to complain of severe dyspnea, and hypoxia was present on pulse oximetry. Therefore, emergency echocardiography was conducted and it showed restrictive cardiomyopathy along with thrombus in the left atrium. With time, heart failure was aggravated despite intensive management. The result of gastric biopsy revealed amyloid deposits which stained positively with Congo red. On immunohistochemistry study, kappa and lambda chain were present. In addition, kappa chain was significantly elevated in urine and serum on electrophoresis. Although the patient was finally diagnosed as having primary gastric amyloidosis with restrictive cardiomyopathy, her general condition rapidly deteriorated and died at 12th hospital day. When obscure gastric lesion is encountered, performing gastric biopsy is strongly recommended since it be primary gastric amyloidosis. Herein, we present an unusual case of primary gastric amyloidosis.
一名53岁女性因上腹部不适和乏力入院。入院时实验室检查显示轻度贫血和蛋白尿。食管胃十二指肠镜检查发现明显的黏膜萎缩、弥漫性结节和颗粒样外观,黏膜质脆。对胃窦和胃体进行了活检。次日,患者开始诉说严重呼吸困难,脉搏血氧饱和度显示存在低氧血症。因此,进行了急诊超声心动图检查,结果显示为限制型心肌病伴左心房血栓形成。随着时间推移,尽管进行了强化治疗,心力衰竭仍加重。胃活检结果显示刚果红染色阳性的淀粉样沉积物。免疫组织化学研究显示存在κ链和λ链。此外,电泳显示尿液和血清中的κ链显著升高。尽管该患者最终被诊断为原发性胃淀粉样变性伴限制型心肌病,但其一般状况迅速恶化,于住院第12天死亡。当遇到不明原因的胃部病变时,强烈建议进行胃活检,因为可能是原发性胃淀粉样变性。在此,我们报告一例不寻常的原发性胃淀粉样变性病例。