Jejunal Amyloidosis: A Rare Cause of Severe Gastrointestinal Bleeding

Primary Localized Amyloidosis of the Intestine: A Pathologist Viewpoint

“…The mean age at presentation was 62 ± 3.7 years (small intestine) and 65.1 ± 3.9 years (colorectum) [ 12 , 15 - 39 ]. In the small intestine, the main symptoms were abdominal pain [ 28 , 33 , 34 , 36 ] and intestinal obstruction (33%) [ 29 , 31 , 35 ], followed by diarrhea and vomiting (16%) [ 28 , 32 ], bleeding [ 30 , 36 ], malabsorption [ 32 ], and constipation [ 34 ]. In the colorectum, the main symptoms were rectal bleeding (37%) [ 15 , 20 - 23 ], followed by abdominal pain (11%) [ 15 , 20 ], anemia [ 18 ], intestinal obstruction [ 16 ] and weight loss [ 15 ].…”

“…In the small intestine, the most common endoscopic findings were thickening of the intestinal wall (18%) [ 28 - 32 , 39 ], and polypoid mucosal protrusions (11%) [ 37 - 39 ], followed by mucosal ulceration (7%) [ 34 , 36 ], friability and nodularity of the mucosa (7%) [ 30 , 36 ], and wall perforations (3%) [ 33 ]. In the colorectum, polypoid masses with luminal narrowing were the most common endoscopic finding (29%) [ 12 , 15 - 18 , 21 ], followed by mucosal ulcerations (solitary or multiple ulcers) (14%) [ 20 , 22 , 25 - 27 ] or and thickening of the wall (7%) [ 20 , 27 ], nodularity and friability of the mucosa (2%) [ 23 , 24 , 26 ] and perforation (3%) [ 20 ].…”

“…When the deposition of the amyloid protein occurs predominantly in the perivascular and interstitial spaces, it usually produces diffuse thickening of the intestinal wall rather than polypoid or tumor-like lesions [20,27,28,31,32]. Elevations or localized tumor-like lesions may reflect the presence of nodular aggregates of plasma cells locally producing abundant amyloid proteins, which usually originate from AL protein produced by local aggregates of plasma cells involving the mucosa, submucosa or the muscle layer [15,17,21,30,37]. Fine granular lesions or mucosal erosion or ulcerations are commonly associated with the deposition of AA amyloidosis [18,20,34].…”

“…The involvement of the neural elements results in altered bowel motility and chronic ileus [ 49 ]. Hemorrhage (melena and bleeding per rectum) is reasoned to bowel ischemia, hemorrhagic necrosis of the mucosa, damage of the vessel walls, and abnormal platelet aggregation [ 30 ].…”

“…In the small intestine, the most common endoscopic findings were thickening of the intestinal wall (18%) [28][29][30][31][32]39], and polypoid mucosal protrusions (11%) [37][38][39], followed by mucosal ulceration (7%) [34,36], friability and nodularity of the mucosa (7%) [30,36], and wall perforations (3%) [33].…”