A rare gastrointestinal manifestation of systemic primary amyloidosis

摘要

Symptomatic gastrointestinal (GI) amyloidosis is a rare manifestation of systemic amyloid light chain (AL) amyloidosis. Further, the presentation of primary GI amyloidosis without previously diagnosed systemic amyloidosis is exceptionally rare. We describe a case of a patient presenting with abdominal pain, nausea, vomiting, and weight loss later to be diagnosed with localized GI amyloidosis from underlying multiple myeloma. Unfortunately, the GI disease was insurmountable leading to her death. Amyloidosis exerts its pathology at the level of the mucosa, soft tissue, nerves, and vascular supply of the GI tract. No specific treatments for the GI complications of amyloidosis are available and supportive measures are universally employed. GI amyloidosis is not only infrequently amenable to systemic and symptomatic treatment, but has a negative impact on both quality of life and survival.