Flashcards
Amyloidosis
Cards in this deck (9)
- 1What is amyloidosis? How is amyloidosis classified?Amyloidosis is the build-up of amyloid protein in extracellular spaces between cells.
Amyloidosis is classified as being local or systemic as well as acquired or inherited. - 2Name the five major classifications of amyloidosis1. Acquired AA amyloidosis
2. Acquired AL amyloidosis
3. Acquired AB2M amyloidosis
4. Acquired ATTR amyloidosis
5. Hereditary ATTR amyloidosis - 3How is Acquired AA amyloidosis caused, what is the precursor and how does it manifest?Acquired AA amyloidosis is caused by a chronic inflammatory disease such as rheumatoid arthritis or tuberculosis. This results in serum AA protein levels in the blood as a result of macrophage secretion of IL1 and IL6 acting on hepatocytes. Serum AA protein (SAA) is the precursor. It manifests as proteinuria and loss of renal function.
- 4How is Acquired AL amyloidosis caused, what is the precursor and how does it manifest?Acquired AL amyloidosis results from plasma cell dyscrasia as a result of DNA damage. Kappa and lambda immunoglobulin chains produced by plasma cells are the precursor. Acquired AL amyloidosis has diverse clinical characteristics including cardiomyopathy, carpal tunnel syndrome and neuropathy.
- 5How is Acquired AB2M amyloidosis caused, what is the precursor and how does it manifest?Acquired AB2M amyloidosis is caused by chronic dialysis as a result of renal failure with high levels of B2-microglobulin precursor. It manifests as carpal tunnel syndrome and problematic joints.
- 6How is Acquired ATTR amyloidosis caused, what is the precursor and how does it manifest?Acquired ATTR amyloidosis is caused by old age (over 80 usually) with wild-type TTR (transthyretin) as a precursor. It manifests as a slowly progressive cardiomyopathy.
- 7How is hereditary ATTR amyloidosis caused, what are the precursors and how does it manifest?Hereditary ATTR amyloidosis is inherited as one of over 80 point mutations in the gene encoding the TTR protein. Its precursor is a mutated TTR protein (transthyretin) and it manifests as peripheral and autonomic neuropathy but can also show cardiac, renal and ocular symptoms.
- 8How does amyloid deposition affect the following organs? Heart Kidneys Brain Skin GI tractHeart- amyloid deposition between cardiocytes puts pressure on parenchymal cells, resulting in cardiac atrophy which can lead to heart failure.
Kidneys- amyloid deposition in blood vessels can increase permeability, resulting in proteinuria and kidney failure
Brain- haemorrhage
Skin- thickening and waxy appearance
GI tract- increased permeability causing severe diarrhoea - 9Which stains are used to visualise amyloid deposits?H&E Stain (hematoxylin and eosin)
Congo Red stain
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- Flashcards on Amyloidosis, created by JodieS on 04/05/2015.
- Created by
- JodieS · May 2015
- Updated
- Feb 19, 2016
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