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Generalized amyloidosis from beta 2-microglobulin, with caecal perforation after long-term haemodialysis
1Pathologisches Institut der Universität, Bonn, Federal Republic of Germany.
Abstract:
A 73-year-old man with chronic renal failure of undetermined aetiology had received haemodialysis for 12 years when he died of acute purulent peritonitis due to caecal perforation. Amyloid deposits detected in a cystic bone lesion in the left hip had caused a pathological fracture 17 days before death. At autopsy, extensive amyloid deposits were found in the osteoarticular system, in the cartilaginous surface and the capsular tissue of joints, ligaments, vertebral discs and bone. In addition, vascular amyloid deposits were diagnosed in the heart, kidneys, testes, lungs, skin and in the gastrointestinal tract. A special feature of this case were interstitial amyloid deposits forming a fine-meshed structure in the myocardium and plate-like deposits in the gastrointestinal tract. Immunohistochemically, all these deposits reacted strongly with antibody to human beta 2-microglobulin but showed no reaction with antibodies to AA, A-lambda, A-kappa and AF. The present case demonstrates that extra-osteoarticular manifestations of AB-amyloidosis can cause serious complications.
Insights
This case study highlights beta 2-microglobulin amyloidosis (AB-amyloidosis) in a dialysis patient. Extensive amyloid deposits outside the bones caused severe complications, including pathological fracture and fatal peritonitis.
Area of Science:
- Nephrology
- Rheumatology
- Pathology
Background:
- A 73-year-old male on long-term hemodialysis for chronic renal failure presented with pathological bone fracture.
- The patient subsequently died from acute purulent peritonitis secondary to caecal perforation.
Observation:
- Autopsy revealed extensive amyloid deposits throughout the osteoarticular system, including joints, ligaments, and bone.
- Vascular amyloid infiltration was noted in multiple organs: heart, kidneys, testes, lungs, skin, and gastrointestinal tract.
- Unique interstitial myocardial and plate-like gastrointestinal amyloid deposits were observed.
Findings:
- Immunohistochemistry confirmed amyloid deposits were composed of human beta 2-microglobulin (AB-amyloidosis).
- Deposits did not react with antibodies for AA, A-lambda, A-kappa, or AF amyloid types.
- The findings indicate a rare manifestation of beta 2-microglobulin amyloidosis.
Implications:
- This case underscores the potential for significant extra-osteoarticular complications of AB-amyloidosis.
- It highlights the importance of considering amyloidosis in patients with chronic renal failure and unexplained symptoms.
- Early recognition and management of AB-amyloidosis may prevent severe systemic complications.