University of South Florida Morsani College of Medicine Tampa, FL
Heather Gosnell, DO1, Kristen Tessiatore, MD1, Sonya Bhaskar, MD2, Richard Pearce, MD2, Edward B.. O'Neill, MD3, Scott Manski, MD2 1University of South Florida Morsani College of Medicine, Tampa, FL; 2University of South Florida, Tampa, FL; 3Tampa General Hospital / University of South Florida, Tampa, FL
Introduction: AL amyloidosis (ALA) is due to free light chain (FLC) deposition from a plasma cell dyscrasia, such as multiple myeloma (MM). FLC deposition in the GI tract can lead to vascular mucosal friability and bleeding, malabsorption syndromes, dysmotility, and protein-losing enteropathy (PLE). Biopsy proven and clinically relevant disease is seldom seen, occurring in 8% and 1% of patients, respectively. Here we present a case of significant PLE from ALA.
Case Description/Methods: A 49 year-old male with a prior cholecystectomy presented with generalized weakness, intermittent post-prandial epigastric pain, anorexia, and a 15 lb unintentional weight loss. Two months prior, he presented to an outside hospital for similar symptoms, and was diagnosed with new onset diabetes and hypothyroidism. Initial bloodwork was notable for multiple metabolic derangements and vitamin deficiencies. Celiac serologies and infectious stool testing were negative. Urine studies demonstrated nephrotic range proteinuria. Kappa-Lambda FLC ratio was elevated. CTA and MRI abdomen/pelvis showed bowel wall thickening, hyperenhancement and vasa recta engorgement in the proximal jejunum, ascending and transverse colon. Cardiac MRI findings were compatible with a diffuse infiltrative process. EGD revealed diffusely erythematous and friable gastric and duodenal mucosa, while colonoscopy showed diffuse mucosal granularity and friability with contact oozing throughout the colon. Biopsies with congo red histochemical stain were positive for amyloid deposition in the stomach, duodenum, terminal ileum, colon and rectum. Gastric biopsies were also positive for H. pylori. Renal and bone marrow biopsies confirmed the diagnosis of kappa-restricted MM. He remains on chemotherapy with Dara-CyborD (daratumumab, cyclophosphomide, bortezomib, dexamethasone) and completed quadruple therapy for H. pylori gastritis. TPN was initiated due to ongoing protein-calorie malnutrition from PLE.
Discussion: This is a unique case of a patient with multiple myeloma with multiorgan system involvement, including gastrointestinal manifestations with diffuse GI tract infiltration on histology. Clinically significant GI manifestations are exceedingly uncommon, presenting in 1% of patients with ALA. Treatment typically involves initiating chemotherapy for the underlying MM and possible bone marrow transplant, replenishing vitamin deficiencies, dietary modifications, symptom management for nausea, vomiting and dysmotility.
Figure: Figure 1: (1A) Diffuse, moderately erythematous mucosa without active bleeding in the second portion of the duodenum. (1B) Diffuse villous blunting and nonspecific erythema in the terminal ileum. (1C-D) Diffuse, patchy erythematous mucosa, mucosal granularity and friability with contact oozing was found in the entire colon. These are representative images from the cecum (1C) and transverse colon (1D). (1E) H&E staining of gastric tissue demonstrating pale pink, glassy-appearing amyloid deposition with classic "cracking artifact". (1F) Congo red staining of gastric biopsies demonstrating pink extracellular amyloid deposition, and again under polarized light (1G) with classic “apple-green birefringence”.
Note: The table for this abstract can be viewed in the ePoster Gallery section of the ACG 2024 ePoster Site or in The American Journal of Gastroenterology's abstract supplement issue, both of which will be available starting October 27, 2024.
Disclosures:
Heather Gosnell indicated no relevant financial relationships.
Kristen Tessiatore indicated no relevant financial relationships.
Sonya Bhaskar indicated no relevant financial relationships.
Richard Pearce indicated no relevant financial relationships.
Edward O'Neill indicated no relevant financial relationships.
Scott Manski indicated no relevant financial relationships.
Heather Gosnell, DO1, Kristen Tessiatore, MD1, Sonya Bhaskar, MD2, Richard Pearce, MD2, Edward B.. O'Neill, MD3, Scott Manski, MD2. P0712 - Protein Losing Enteropathy in Biopsy Proven Gastrointestinal AL Amyloidosis, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.
