title: Rare presentation of primary (AL) amyloidosis as gastrointestinal hemorrhage without systemic involvement.
abstract: We are reporting a rare case of a patient with primary (AL) amyloidosis presenting with an acute non-variceal upper gastrointestinal hemorrhage in the absence of other systemic involvement. The case report involves a 58-year-old woman with significant cardiac history and hereditary blood disorder who came in complaining of abdominal pain and coffee-ground emesis for two days. Computed tomography (CT) scan of the abdomen and pelvis with contrast revealed segmental wall thickening of the proximal jejunum with hyperdense, heterogenous luminal content. Similar findings were evident in the left lower small bowel region, suspicious for small bowel hematoma and the possibility of intraluminal clots. Esophagogastroduodenoscopy performed post resuscitation showed punctate, erythematous lesions throughout the stomach as well as regions of small bowel mucosa that appeared scalloped, ulcerated, and hemorrhaged on contact. Despite initial treatment for immunostain-positive focal cytomegalovirus gastritis, follow-up esophagogastroduodenoscopy after two months continued to demonstrate friable and irregular duodenal mucosa hinting at a different underlying etiology. Pathology reports from analyses of biopsy samples highlighted infiltration and expansion of the lamina propria and submucosa. Subsequent staining with congo red/crystal violet and appropriate subtyping established the diagnosis of AL (kappa)-type amyloidosis. The significance of this case lies in the fact that our patient did not have the typically seen diagnostic systemic involvements-namely of heart and kidneys-usually seen in primary (AL) amyloidosis patients. It was the persistent endoscopic findings and biopsy results which gave clues to the physicians regarding the possibility of an abnormal protein-deposition entity.
authors.affiliation: Mohammad F Ali, Department of Internal Medicine, Winthrop University Hospital, Mineola, NY 11501, United States.
authors.forename: Mohammad F
authors.initials: MF
authors.lastname: Ali
authors.affiliation: Mohammad F Ali, Department of Internal Medicine, Winthrop University Hospital, Mineola, NY 11501, United States.
authors.forename: Anik
authors.initials: A
authors.lastname: Patel
authors.affiliation: Mohammad F Ali, Department of Internal Medicine, Winthrop University Hospital, Mineola, NY 11501, United States.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Muller
authors.affiliation: Mohammad F Ali, Department of Internal Medicine, Winthrop University Hospital, Mineola, NY 11501, United States.
authors.forename: David
authors.initials: D
authors.lastname: Friedel
keywords: AL amyloidosis; Amyloid deposition; Endoscopic biopsy; Endoscopic finding; Gastric/intestinal mucosa; Gastrointestinal hemorrhage; Mucosal inflammation; Primary amyloidosis; Upper gastrointestinal bleeding
references.citation: Eur J Echocardiogr. 2009 Dec;10(8):iii23-33
references.pmid: 19889655
references.citation: J Gastroenterol Hepatol. 2002 May;17(5):623-4
references.pmid: 12084040
references.citation: Mayo Clin Proc. 1993 Aug;68(8):763-7
references.pmid: 8331978
references.citation: Semin Hematol. 1995 Jan;32(1):45-59
references.pmid: 7878478
references.citation: J Clin Gastroenterol. 2001 Feb;32(2):161-3
references.pmid: 11205655
references.citation: Scand J Gastroenterol. 2009;44(6):708-11
references.pmid: 19242859
references.citation: Hepatogastroenterology. 1992 Jun;39(3):267-9
references.pmid: 1505902
references.citation: WMJ. 2008 Feb;107(1):40-3
references.pmid: 18416369
references.citation: Am J Gastroenterol. 2008 Mar;103(3):776-87
references.pmid: 18076735
references.citation: Gastrointest Endosc. 1990 Jan-Feb;36(1):10-4
references.pmid: 2311879
references.citation: Hellenic J Cardiol. 2010 Nov-Dec;51(6):552-7
references.pmid: 21169191
references.citation: Blood. 1992 Apr 1;79(7):1817-22
references.pmid: 1558973