Large Gastric Mucosal Tear Secondary to Gastric AL Amyloidosis

Abstract

Purpose: Gastric symptoms from Amyloid Light-chain (AL) amyloidosis are rare. This is a patient with hematemesis found to have a massive gastric mucosal tear secondary to gastric AL amyloidosis. Case Report: A 47-year-old woman presented with hematemesis, epigastric and chest pain. She had no prior symptoms, no known past medical, surgical, or family history. She denied use of medications. Vital signs were normal. She had a right eye ecchymosis (post-emesis), chest and epigastric tenderness, but no crepitus. Esomeprazole drip was initiated. Hemoglobin was 9.0 g/dL, platelets 222 K/uL, total protein 9.5 g/dL, albumin 3.5 g/dL, and INR was 1.2. Chest x-ray was unremarkable. Upper endoscopy revealed a large deep mucosal tear along the lesser curvature of the gastric body oozing blood with friable mucosa (Figure 1A). Chest CT showed a gastric submucosal hematoma, and no perforation (Figure 1B). She had no further major events. Repeat endoscopy showed a nodular mucosa with linear scarring. Air insufflation caused superficial mucosal tearing in the gastric body and antrum (Figure 1C). Biopsy results showed submucosal amorphous material deposition, and positive Congo Red stain consistent with amyloid (Figure 1D). Results of serum electrophoresis and bone marrow biopsy revealed a diagnosis of AL amyloidosis with IgG Lambda multiple myeloma. She was treated with bortezomib, cyclophosphamide, and dexamethasone.Figure 1: A. Retroflexed view of the lesser curve gastric hematoma / tear (arrow). B. CT-gastric hematoma (arrow). C. Tearing on insufflation. D. Biopsy-Congo Red stain.Discussion: Incidence of AL amyloidosis in the U.S. is 6-10 cases per million personyears, with 10-15% of multiple myeloma patients having AL amyloid. Only 1% have symptoms from gastric involved AL amyloidosis. Patients can present with peri-orbital pinch purpura. Endoscopic features include ulcerations, granular and friable mucosa, irregular gastric folds, submucosal hemorrhage, and hematomas. Bleeding in AL amyloidosis is due to capillary fragility from amyloid deposition, coagulation factor deficiency, especially low factor X, and abnormal platelets. The goal of therapy is to suppress the synthesis of light chains by treating the underlying disorder.