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Primary amyloidosis presenting as small bowel encapsulation.

Jennifer Jones1, Guido VanRosendaal, Cynthia Cleary

  • 1Department of Medicine, Division of Gastroenterology, Dalhousie University, Halifax, Nova Scotia. joneskenyon@yahoo.ca

Canadian Journal of Gastroenterology = Journal Canadien De Gastroenterologie
|April 1, 2004
PubMed
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This case report details a rare instance of primary amyloidosis causing small bowel obstruction via encapsulating peritonitis. It highlights the diagnostic challenges and unique presentation of amyloidosis.

Area of Science:

  • Gastroenterology
  • Nephrology
  • Oncology

Background:

  • Amyloidosis involves extracellular deposition of fibrillar proteins, leading to diverse clinical presentations based on organ involvement.
  • Primary amyloidosis, often associated with plasma cell dyscrasias, typically affects organs like the kidneys, heart, and nerves.

Observation:

  • A 62-year-old man presented with small bowel obstruction due to a unique membrane encasing the small bowel, diagnosed as encapsulating peritonitis due to amyloidosis.
  • Initial biopsies were inconclusive, but duodenal biopsies confirmed primary amyloidosis, with subsequent development of nephrotic syndrome and renal amyloidosis.

Findings:

  • Histopathology revealed Congo red-positive, apple-green birefringent material consistent with amyloid deposition.
  • The patient had a monoclonal paraprotein (Immunoglobulin G lambda) and mild bone marrow plasmacytosis, indicative of an underlying plasma cell disorder.

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  • Despite initial asymptomatic recovery, progressive renal involvement necessitated high-dose chemotherapy and autologous stem cell transplant.
  • Implications:

    • This case represents the first documented instance of primary amyloidosis presenting as small bowel obstruction secondary to encapsulating peritonitis.
    • It underscores the importance of considering amyloidosis in unexplained gastrointestinal obstructions, even with atypical presentations.
    • The case highlights the potential for multi-organ involvement in primary amyloidosis and the need for comprehensive diagnostic workups.