[Two autopsy cases with systemic amyloidosis--case 11/2010].
METHODS
A 37-year old patient was admitted with upper abdominal pain, vomiting and diarrhea. A 38-year-old patient was admitted for liver failure.


METHODS
Case 1 was diagnosed with an AL amyloidosis due to deposition of the immunoglobulin light chain kappa in all tissues analyzed. In the bone marrow plasma cells were increased to 20-30%. Case 2 suffered from AA amlyoidosis secondary to familial mediterranean fever and underwent dialysis treatment for years. He was positive for hepatitis B and C.


METHODS
Patient 1 developed refractory nephrotic syndrome and low blood pressure. During hemodialysis circulatory failure occured and she died during resuscitation. In patient 2 a flare of chronic hepatitis B was found and treated with antiviral therapy. He was referred to ICU for rectal bleeding and developed pulmonary arrest. After resuscitation he died because of lactate acidosis and refractory circulatory failure. Both cases were subjected to autopsy.


CONCLUSIONS
The vast majority (90%) of amyloidoses are due to acquired AA or AL amyloidosis. Prognosis remains poor, in particular when cardiac and vascular involvement occurs.
Institut für Pathologie, Universitätsklinikum Tübingen, Tübingen.
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S
Schott
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Artunc
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M
Haap
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Mackensen-Haen
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Fend
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R
Riessen
D000328:Adult; D000686:Amyloidosis / Q000473:pathology*; D001344:Autopsy; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D005753:Gastric Mucosa / Q000473:pathology*; D005773:Gastroscopy; D019694:Hepatitis B, Chronic / Q000473:pathology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood; D007145:Immunoglobulin kappa-Chains / Q000097:blood; D007146:Immunoglobulin lambda-Chains / Q000097:blood; D007413:Intestinal Mucosa / Q000473:pathology; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000473:pathology; D008099:Liver / Q000473:pathology; D017093:Liver Failure / Q000473:pathology; D008297:Male; D009101:Multiple Myeloma / Q000473:pathology*; D009206:Myocardium / Q000473:pathology; D010265:Paraproteinemias / Q000473:pathology
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains