title: Globular amyloid deposits isolated to the small bowel: a rare association with AL amyloidosis.
abstract: We describe a 55-year-old man with isolated duodenal and jejunal amyloidosis producing rare endoscopic and histologic findings. The patient had no specific gastrointestinal complaints but underwent esophagogastroduodenoscopy and colonoscopy because of progressive microcytic anemia. Endoscopy revealed multiple polyps, some filiform and measuring up to 3 cm in length, in the duodenum and proximal jejunum. Microscopically, the polyps resulted from amyloid deposition, predominantly within the submucosa, but also focally involving muscularis mucosae and lamina propria. The amyloid formed multiple globular submucosal deposits with a lamellated appearance reminiscent of corpora amylacea; linear amyloid deposition was also present in a perivascular distribution and within the overlying mucosa. Immunophenotyping confirmed AL amyloidosis with lambda immunoglobulin light chain restriction. There was no clinical evidence of visceral amyloidosis. The source of lambda light chain production was unclear as bone marrow biopsy and multiple gastrointestinal biopsies revealed normal numbers of polyclonal plasma cells. Further, immunoglobulin-free light chain assay was normal, as were serum and urine protein electrophoreses with immunofixation. This endoscopic presentation of isolated small bowel polyposis is an uncommon association with AL amyloidosis and to our knowledge this represents the first case of globular gastrointestinal amyloidosis resulting from AL amyloid.
authors.affiliation: Division of Anatomic Pathology, Department of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Patrick R
authors.initials: PR
authors.lastname: Hemmer
authors.forename: Mark D
authors.initials: MD
authors.lastname: Topazian
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
authors.forename: Susan C
authors.initials: SC
authors.lastname: Abraham
mesh_terms: D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications / Q000276:immunology / Q000473:pathology*; D004378:Duodenal Diseases / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism; D044483:Intestinal Polyposis / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D007579:Jejunal Diseases / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged
chemical_list: D000682:Amyloid; D007147:Immunoglobulin Light Chains