Abstract

INTRODUCTION: Gastrointestinal amyloidosis (GIA) ensues from the deposition of insoluble extracellular protein fragments. GIA is most frequently associated with chronic inflammatory disorders (AA amyloidosis), plasma cell dyscrasias like multiple myeloma (AL amyloidosis), and end-stage renal disease (Beta-2 amyloidosis). CASE DESCRIPTION/METHODS: A 60-year-old female with a history of diastolic heart failure, light-chain multiple myeloma complicated with systemic amyloidosis and CKD stage III presented with acute epigastric abdominal pain associated with vomiting and non-bloody diarrhea that started a week after she had chemotherapy with bortezomib and elotuzumab. She was on chronic steroid and PPi therapy. On exam, she was noted to have tachycardia, dry mucous membranes, macroglossia and epigastric tenderness with no rebound. Her labwork showed a drop in hemoglobin from 8 g/dL to 5.9 g/dL in a period of 2 days and a platelet count of 48000/mL. Coagulation profile, liver tests and kidney tests were all normal. In the ED, her stool was positive for occult blood. CECT abdomen showed diffuse small and large bowel wall thickening, including the stomach and duodenum. Gastroscopy showed diffuse discontinuous congestion, friability, and ulceration of the mucosa. Clots overlying ulcers were removed with forceps revealing mild oozing only. The gastric biopsy revealed mucosal cells with extracellular amorphous deposits, staining positive for congo red stain, consistent with amyloid. She was started on palliative chemotherapy given her multi-system involvement of malignancy. DISCUSSION: GIA involves the buildup of insoluble proteins, most commonly in the second part of duodenum which impedes the normal organ structure and function. Weight loss and GI bleeding are the most common presentations of GIA. Bleeding is a sequela of mucosal lesions, wall ischemia, or vascular friability. The endoscopic appearance of amyloidosis is nonspecific. The mucosa may have a finely granular appearance, polypoid protrusions, erosions, ulcerations, and thickening of the wall. Tissue biopsy is required to make the diagnosis of GI amyloidosis. Treatment depends on a sufficient suppression of the predisposing inflammatory disorder, or malignancy, along with supportive therapy. The prognosis of patients with AL amyloidosis is worse with GI involvement.

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