Systemic Amyloidosis Manifested by Gastric Outlet Obstruction
An 80-year-old female presented to Division of Gastroenterology, Department of Internal Medicine, Chung-Ang Medical Center with a complaint of a 2-week history of vomiting. She was a nonsmoker, did not consume alcohol and had no previously diagnosed disease. The patient complained of epigastric pain and had mild epigastric tenderness. She was admitted to the hospital for evaluation and treatment. Laboratory data revealed anemia (9.5 g/dL), but she had a white blood cell and a platelet count within normal ranges. Serum chemistry indicated a hypoproteinemia and hypoalbuminemia (total protein, 5 g/dL; albumin, 1.7 g/dL), but without abnormal liver or renal function. An esophagogastroduodenoscopy showed erythematous and edematous mucosa on the antrum with pyloric stenosis (). A computed tomography scan with contrast revealed diffuse wall thickening in the antrum of the stomach with partial gastric outlet obstruction, and without either perigastric fat infiltration or regional lymphadenopathy (). Histopathologic examination showed the deposition of amorphous, homogeneous, and acidophilic material in the gastric mucosal. The amyloidal protein was proven by a positive Congo red stain result (). Immunohistochemistry performed on biopsy results was C-kit (-), SMA (-), CD34 (-), S-100 (-), and Ki-67 (<1%).\nThe existence of a gastric amyloid tumor suggested systemic amyloidosis. Therefore, we did blood examinations, rapid plama reagin, rheumatoid factor, urinalysis, chest-abdominal simple X-ray examinations, skull and pelvic series, rectal biopsy with sigmoidoscopy, electrocardiography, and cardiac ultrasonography. A serum and urine immunfixation electrophoresis showed abnormal band of restriction in lambda light chain, suggesting monoclonal component. The cardiac ultrasonography had shown a small amount of pericardial effusion and diastolic dysfunction grade I. Small amounts of both pleural effusion and ascites were noted on the computed tomography scan. Other examinations revealed no specific finding. She was diagnosed with AL type systemic amyloidosis that presented as gastric outlet obstruction.\nDuring her admission, she developed symptoms, repeated bile color vomiting, malabsorption, systemic edema, dyspnea, and melena. On hospital day 14, a follow-up esophagogastroduodenoscopy showed progressed infiltration type gastric outlet obstruction and a single hole was noted on the antrum of nasogastric tube insertions site (). During the esophagogastroduodenoscopy, the scope did not pass through the pylorus. She refused further evaluation and treatment such as chemotherapy or surgery. Instead, she was managed conservatively with total parenteral nutrition therapy and intravenous proton pump inhibitor. She died of disease progression after 34 hospital days.