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• W2922381189 abstract "Introduction: Symptomatic upper GI involvement is rarely seen in amyloidosis. It is even rarer to see it form of non caseating granulomas on histopathology. We present the first reported case of AL amyloidosis manfesting with upper GI symptoms with non caseating granulomas seen on biopsy. Case presentation: A 56 year old African American woman with newly diagnosed heart failure presented to the office with 40 lb weight loss, vomiting, early satiety and progressive anemia. Computed tomography of abdomen showed concentric thickening of the stomach and diffuse mesenteric fat infiltration. She subsequently underwent upper endoscopy which showed nodularity of the gastric body, antrum and duodenal mucosa concerning for malignancy. Biopsies showed scattered non-necrotizing granulomas without any dysplastic cells. Tuberculosis, sacrcoidosis and syphilis were ruled out after further testing. Echocardiography showed ejection fraction of 40% alongwith diastolic dysfunction and deformation parameters consistent with cardiac amyloidosis. Further evaluation of anemia showed an elevated M spike on peripheral blood. Bone marrow revealed hypercellular marrow with 30-60% kappa restricted plasma cells, thereby making the diagnosis of multiple myeloma with subsequent AL Amyloidosis. The patient was subsequently started on cyclophosphamide, bortezomib and dexamethasone and is being followed up. Discussion: AL amyloidosis is the most subtype of systemic amyloidosis. Biopsy proven gastroduodenal involvement of AL amyloidosis is seen in 8% of patients with amyloidosis while symptomatic involvement is even more rare (1%). Presenting features include early satiety, nausea and hematemesis. Endoscopy demonstrates thickening of the gastric and duodenal folds, ulcers with occasional overhanging borders (mimicking carcinoma), and gastroparesis. Histopathology usually shows positive green birefringence seen on Congo red staining. Very rarely, amyloidosis may lead to non caseating granulomas in the involved tissue, however no prior reports exist in literature that describe this finding in the upper gastrointestinal tract. Recently, high-dose chemotherapy with hematopoietic stem cell transplantation has shown encouraging results for AL amyloidosis including improvement in amyloid related organ dysfunction. Conclusion: Although rare, amyloidosis should be kept in the differential diagnosis of patients with granulomatous gastritis especially with concomitant cardiomyopathy and anemia.2716_A Figure 1. Narrow band imaging of granular appearing gastric mucosa2716_B Figure 2. Granular appearing duodenal mucosa" @default.
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• W2922381189 date "2018-10-01" @default.
• W2922381189 modified "2023-09-27" @default.
• W2922381189 title "This Looks Malignant but Is It Really Cancer? A Case of Symptomatic Systemic Amyloidosis Presenting as Granulomatous Gastroduodenitis" @default.
• W2922381189 doi "https://doi.org/10.14309/00000434-201810001-02715" @default.
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