Selective Massive Amyloidosis of Small Intestine

Griffel, Benjamin

doi:10.1001/archsurg.1975.01360080081015

Cited by 28 publications

(10 citation statements)

References 18 publications

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“…Among patients with systemic amyloidosis, gastrointestinal involvement occurs in at least 70% 6 ; in more sensitive autopsy studies with examination of fullthickness sections from stomach, small bowel, and colon, nearly all patients have involvement of some layer of the bowel wall regardless of whether the amyloidosis is of AA, AL, or b 2 -microglobulin types. 26 Less commonly, amyloid deposition is isolated to one or more organs of the gastrointestinal tract, including reports of patients with amyloid confined to the stomach, 5,7,12 small bowel, 4,8,12,13,15,27 or colorectum. 12,17,24,30,33 Rarely, gastrointestinal amyloid presents as tumoral masses causing obstruction, 4 hematemesis, 5 and clinical confusion with adenocarcinoma.…”

mentioning

confidence: 99%

Globular Amyloid Deposits Isolated to the Small Bowel

Hemmer¹,

Topazian²,

Gertz

et al. 2007

American Journal of Surgical Pathology

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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.

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confidence: 99%

Globular Amyloid Deposits Isolated to the Small Bowel

Hemmer¹,

Topazian²,

Gertz

et al. 2007

American Journal of Surgical Pathology

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“…In the small and large bowel this manifests as malabsorption syndromes,5 , 6 and in the oesophagus and stomach, it can produce dysmotility and gastroparesis respectively. Patients can also present with obstruction7 , 8 or perforation 9 , 10. Uniquely, our case has had evidence of symptomatic oesophageal as well as gastric dysmotility.…”

Section: Discussionmentioning

confidence: 66%

A rare cause of dysphagia and gastroparesis

Basavaraju

Mansour²,

Barnes³

et al. 2009

Case Reports

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An 82-year-old woman was admitted with severe vomiting and progressive dysphagia mainly to solids. She gave a 3-month history of increasing heartburn, vomiting, tiredness, lethargy, anorexia and 13 kg weight loss. Her past medical history was unremarkable and she was a non-smoker. Physical examination revealed evidence of significant weight loss and dehydration only. Gastroscopy revealed mild oesophagitis, tongues of Barrett oesophagus and mild antral gastritis. CT scan of the thorax and abdomen was normal. Unfortunately her condition deteriorated rapidly and she died from aspiration pneumonia. Postmortem examination revealed thickening of the muscular wall of lower oesophagus and pylorus, but without any malignancy. The histological assessment of the oesophageal as well as gastric biopsies confirmed the diagnosis of gastrointestinal amyloidosis accounting for her symptoms of dysphagia and vomiting respectively.

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“…Amyloidosis may also mimic small-bowel tumors [13][14][15] and, rarely, may be associ ated with inflammatory bowel disease [16,17], Perineural deposits may interfere with motility de spite the concept that each segment of intestine can act as a pacemaker; it is possible, as Bass et al [18] suggest ed, that a finite length of bowel is required to promote peristalsis. If this is so , amyloid deposits around nerve plexi would prevent the myoelectrical control of the involved intestinal segment and result in a functional obstruction.…”

Section: Discussionmentioning

confidence: 99%

Management of Amyloidosis Presenting as Intestinal Obstruction

Gerst¹,

Deshmukh²,

Ziscovici³

et al. 1990

Dig Surg

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A 55-year-old woman presented with symptoms and signs of intestinal obstruction. At exploratory laparotomy, neither an extra- nor an intraluminal obstructing lesion was found. However, there was a thickened midileal small-bowel segment with an abnormal mesentery and a significant proximal dilatation. Wide resection of the involved segment was performed. Histology of the resected specimen yielded the diagnosis of amyloidosis. The management of functional obstruction (pseudo-obstruction) of the small intestine caused by amyloidosis is discussed and the literature on this subject is reviewed.

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Selective Massive Amyloidosis of Small Intestine

Cited by 28 publications

References 18 publications

Globular Amyloid Deposits Isolated to the Small Bowel

Globular Amyloid Deposits Isolated to the Small Bowel

A rare cause of dysphagia and gastroparesis

Management of Amyloidosis Presenting as Intestinal Obstruction

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