Korean J Gastroenterol.  2015 Oct;66(4):227-230. 10.4166/kjg.2015.66.4.227.

A Case of Primary Gastric Amyloidosis with Fulminant Heart Failure

• Hong S
• Chang YW
• Byun JK
• Kim MJ
• Chae JM
• Park SH
• Oh CH
• Park YK

• KMID: 2373346
• DOI: http://doi.org/10.4166/kjg.2015.66.4.227

Abstract

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.

Keyword

Amyloidosis; Stomach; Heart failure

MeSH Terms

Amyloidosis/complications/*diagnosis/pathology
Endoscopy, Digestive System
Female
Heart Atria/diagnostic imaging
Heart Failure/complications/*diagnosis
Humans
Immunoglobulin kappa-Chains/blood/urine
Immunoglobulin lambda-Chains/blood/urine
Immunohistochemistry
Magnetic Resonance Imaging
Middle Aged
Stomach Diseases/complications/*diagnosis/pathology
Thrombosis/diagnosis/diagnostic imaging
Tomography, X-Ray Computed
Ultrasonography
Immunoglobulin kappa-Chains
Immunoglobulin lambda-Chains

Figure

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  Fig. 1. Endoscopic findings of the stomach. (A) On greater curvature and (B) lesser curvature of the stomach, marked mucosal atrophy and diffuse granular surface appearances are noted along with mucosal friability.

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  Fig. 2. CT scan of the thorax. Two thrombi are present in the right atrium and left atrium appendage (arrows). Pleural effusion is also observed.

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  Fig. 3. Echocardiography. Significant hypertrophy and bright twinkling echogenecity are observed. A thrombus is also noted in the left atrium (arrow).

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  Fig. 4. Cardiac MRI. On delayed enhanced image, abnormal global subendocardial enhancement is shown.

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  Fig. 5. Microscopic findings. (A) Profound eosinophilic masses are noted in lamina propria (H&E, ×100). (B) Congo-red stain reveals pink red deposits (×200). (C) The yellow-green bifringence of the deposits is observed by polarizing microscope (×200).

Reference

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