Intestinal Amyloidosis with Intractable Diarrhea and Intestinal Pseudo-obstruction

Abstract

We report herein a case of intestinal amyloidosis with grave prognosis that caused intractable diarrhea and intestinal pseudo-obstruction, alternately in spite of intensive conservative treatment. A 44-year-old woman was admitted for fever, diarrhea, and crampy abdominal pain which had been continuned during 6 months. Abdomen CT scan showed edematous wall thickening of the small bowel and right colon, and colonoscopic biopsy revealed amyloid deposition in the mucosa. Monoclonal light chains in serum and/or urine were not detected and highly elevated serum amyloid A was shown. In spite of intensive treatment including oral prednisolone and colchicine, diarrhea and intestinal pseudo-obstruction developed alternately, general status rapidly got worsened and died after two months.

Primary Localized Amyloidosis of the Intestine: A Pathologist Viewpoint

“…In all cases, the workup for monoclonal gammopathy and systemic amyloidosis (serum protein electrophoresis and pathological examination of the tissue specimens) was performed. The diagnosis of localized amyloidosis was substantiated by the use of ancillary studies including radiological examination such as abdominal ultrasound, barium enema [ 15 ],whole-body computed tomography [ 34 ], magnetic resonance imaging, positron emission tomography [ 28 ], skeletal surveys, electrocardiography and echocardiography [ 12 , 21 ]. The reported cases did not have any evidence of generalized metabolic complications or extra-intestinal complications of accumulated amyloidosis [ 12 , 15 - 40 ].…”

“…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 ].…”

“…The variations in the gross findings of intestinal amyloidosis may be reasoned to the type and the site of deposition of the amyloid protein. 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].…”

“…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].…”

“…Abdominal pain and intestinal obstruction (33%) [6,28,29,31,[33][34][35][36] Rectal bleeding (37%) [15,[20][21][22][23], followed by abdominal pain (11%) [15,20] and anemia [18] Colonoscopic findings…”