Upper Gastrointestinal Bleeding Due to Amyloidosis in a Patient With Multiple Myeloma
Authors: Karanprit Singh, Prateek Chapalamadugu, Peter Malet
Journal: Clinical Gastroenterology and Hepatology (2016)

A 61-year-old man with a history of multiple myeloma, atrial fibrillation, and cardiac amyloidosis presented with hematemesis and melena. He also reported a 2-week history of ibuprofen use for low back pain. He was recently started on apixaban for atrial fibrillation. Initial biochemical investigations revealed a hemoglobin level of 9.8 g/dL and hematocrit of 30.5%. The patient was administered intravenous pantoprazole at 8 mg/h and 2 U of packed red blood cells. The patient had a massive melenic bowel movement while in the intensive care unit. Repeat biochemical investigations showed a hemoglobin level of 8.3 g/dL and hematocrit of 25%. Two additional units of packed red blood cells were transfused and 1 U of platelets. The hemoglobin and hematocrit subsequently stabilized with no further bloody bowel movements. The patient underwent an esophagogastroduodenoscopy (EGD), which revealed several gastric ulcers located in the fundus and body of the stomach and a gastric ulcer in the distal body of the stomach measuring 10 mm in size (Figure A). No biopsies were taken because there was a concern for bleeding. The patient was placed on oral pantoprazole, 40 mg twice daily, and was instructed to return in 2 weeks to evaluate the gastric ulcers. Repeat EGD in 2 weeks revealed a healing erythematous nodular ulcer in the distal body of the stomach (Figure B). Random biopsies were taken diffusely throughout the stomach. Histopathology revealed diffuse amyloid deposition in the antrum, body, and distal body of the stomach (Figure C). The patient reported no further episodes of gastrointestinal (GI) bleeding. He was instructed to continue proton pump inhibitor therapy and to follow up with his oncologist. Amyloidosis is the deposition of extracellular tissue of fibrils composed of serum proteins. Systemic amyloid deposition can be acquired, as in immunoglobulin light chain amyloidosis, and is commonly caused by an underlying plasma cell dyscrasia, such as multiple myeloma. Chronic inflammatory diseases, such as rheumatoid arthritis and inflammatory bowel disease, can also lead to systemic amyloid deposition. GI disease is present in <10% of patients with immunoglobulin light chain amyloidosis.1Menke D.M. Kyle R.A. Fleming C.R. et al.Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis.Mayo Clin Proc. 1993; 68: 763Google Scholar Common sites of amyloid infiltration, from most common to least common, are the second part of the duodenum, stomach, colorectum, and esophagus.2Tada S. Iida M. Iwashita A. et al.Endoscopic and biopsy findings of the upper digestive tract in patients with amyloidosis.Gastrointest Endosc. 1990; 36: 10Google Scholar GI bleeding is the presenting symptom in 25%–45% of patients with GI tract amyloidosis.3Levy D.J. Franklin G.O. Rosenthal W.S. Gastrointestinal bleeding and amyloidosis.Am J Gastroenterol. 1982; 77: 422Google Scholar It may be caused by ischemia or infarction; vascular friability; or mucosal lesions, such as ulcers, polypoid lesions, erosions, submucosal hematomas, or small mucosal hemorrhages. Gjeorgjievski et al4Gjeorgjievski M. Purohit T. Amin M.B. et al.Upper gastrointestinal bleeding from gastric amyloidosis in a patient with smoldering multiple myeloma.Case Rep Gastrointest Med. 2015; : 1-6Google Scholar report a 92-year-old woman with a history of smoldering multiple myeloma who presented with orthostatic dizziness and melena for a few days. Initial EGD revealed a hemispherical 11 × 6 cm tightly adherent clot attached to the midgastric body that could not be detached despite vigorous endoscopic irrigation and aspiration. After being administered intravenous pantoprazole at 8 mg/h and 2 U of packed red blood cells, a repeat EGD performed 1 day later showed that the clot had mostly dissolved exposing a 2.5 × 2 cm ulcerated mass. Histologic staining of the biopsied mass was consistent with gastric amyloidosis.4Gjeorgjievski M. Purohit T. Amin M.B. et al.Upper gastrointestinal bleeding from gastric amyloidosis in a patient with smoldering multiple myeloma.Case Rep Gastrointest Med. 2015; : 1-6Google Scholar Patients may also present with malabsorption, protein-losing gastroenteropathy, and chronic GI dysmotility manifesting as constipation, nausea, vomiting, and abdominal pain. Tissue biopsy is required to make the diagnosis of GI amyloidosis. Based on GI symptoms, we perform an upper endoscopy and/or a colonoscopy to obtain duodenal or rectal mucosal biopsies in patients with suspected amyloidosis. Endoscopic appearance of amyloidosis is nonspecific. The mucosa may have a fine granular appearance, polypoid protrusions, erosions, ulcerations, and thickening of the wall.2Tada S. Iida M. Iwashita A. et al.Endoscopic and biopsy findings of the upper digestive tract in patients with amyloidosis.Gastrointest Endosc. 1990; 36: 10Google Scholar Treatment of the underlying disease has been associated with regression of GI amyloid.