Fatal gastrointestinal hemorrhage in a paraplegic man with undiagnosed AA (secondary) amyloidosis.
We report a case of a 41-year-old African-American male paraplegic for more than 20 years, who had recurrent infections, renal failure with total urine protein of 840 mg/dL on urine protein electrophoresis and adrenal cortical insufficiency. He died suddenly of massive gastrointestinal (GI) hemorrhage. Autopsy showed clinically undiagnosed systemic amyloidosis involving the kidneys, adrenal cortices, spleen and small blood vessels of most organs and tissues, including those of the gastrointestinal tract. The history and autopsy findings indicated secondary or amyloid A (AA) amyloidosis. Paraplegia was one of the most common causes of secondary amyloidosis decades ago, but has now become unusual in patients with AA amyloidosis. Extensive involvement of GI small vessels was the most likely cause of fatal bleeding. GI amyloid previously has been shown to cause hemorrhage, but a fatal case has not yet been described.
Department of Pathology and Laboratory Medicine, Temple University Hospital, Philadelphia, PA 19140, USA. mabaird16@yahoo.com
Marilyn A
MA
Baird-Howell
John
J
Wurzel
D000328:Adult; D000686:Amyloidosis / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D001808:Blood Vessels / Q000378:metabolism / Q000473:pathology; D004943:Esophagogastric Junction / Q000098:blood supply / Q000378:metabolism / Q000473:pathology; D017809:Fatal Outcome; D006471:Gastrointestinal Hemorrhage / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D006801:Humans; D007678:Kidney Glomerulus / Q000098:blood supply / Q000378:metabolism / Q000473:pathology; D008297:Male; D010264:Paraplegia / Q000150:complications*; D011507:Proteinuria / Q000209:etiology; D000685:Serum Amyloid A Protein / Q000378:metabolism*
D000685:Serum Amyloid A Protein