Gastrointestinal Primary Light Chain Amyloidosis (AL): Safety and Utility of Endoscopic Evaluation and Management of GI Bleeding

Abstract

Introduction: Multiple reports have cited the potential for GIB in patients with AL. We report 2 cases demonstrating the utility and risks of endoscopy in this setting. Case 1: A 50yo F with multiple myeloma (MM) presented with recurrent hematemesis. Multiple gastric mucosal ulcers and submucosal hematomas (SMH) were found during initial EGD [1a]; biopsies confirmed AL. Repeat exam was performed prior to consideration of stem cell transplant (SCT) one year later. On scope insertion, patchy areas of scar with mottled mucosal discoloration were noted [1b]. On scope withdrawal, spontaneous development of multiple enlarging SMHs and mucosal tears were found in the gastric body related to scope +/- barotrauma not amenable to endotherapy [1c]. Clotting parameters were normal. Although no intervention was required, the patient was deemed not to be a candidate for SCT given high bleeding risk and was continued on lenalidomide. Case 2: A 69yo M with MM s/p SCT on therapy for relapse presented with recurrent hematochezia. During colonoscopy, multiple SMHs and ulcers were found with normal intervening mucosa [2]. Although negative on 3 prior occasions, repeat biopsies confirmed AL. Selective APC and endoclips had been tried without success. Bleeding continued and ultimately total colectomy was performed; unfortunately he passed soon after with complications from systemic amyloidosis. Discussion: AL results in abnormal extracellular protein deposition in various organs including the GI tract and is commonly associated with MM (>15%). Overt GIB may occur in ˜40%. Amyloid infiltration of submucosal vessels induces ischemic change and increases vascular fragility as well as rigidity of the intestinal wall, which can lead to tearing with hemorrhage. Common endoscopic findings include diffuse mucosal erosions, ulcerations, and SMHs, which can be massive. SMHs are highly suggestive of AL in the proper clinical setting, although biopsy is still required to confirm diagnosis, which can be challenging. In most reported cases, SMHs were identified on endoscope insertion and were not thought to have resulted from scope-induced trauma or shear forces associated with scope manipulation. However our experience shows that endoscopy can be traumatic and should be considered high risk. Treatment is often difficult and of limited utility. Conclusion: Guidelines for endoscopy in the setting of GI amyloidosis and GIB are lacking and these patients should be approached with caution.Figure 1Figure 2