Amyloidosis

Todayโ€™s path rounds are on ๐š๐ฆ๐ฒ๐ฅ๐จ๐ข๐๐จ๐ฌ๐ข๐ฌ!

๐–๐ก๐š๐ญ ๐ข๐ฌ ๐ข๐ญ?
๐€๐ฆ๐ฒ๐ฅ๐จ๐ข๐๐จ๐ฌ๐ข๐ฌ is caused by a group of diseases that result in high levels of ๐š๐ฆ๐ฒ๐ฅ๐จ๐ข๐ being deposited in the tissues. Amyloid is a protein material that has a characteristic folding pattern, but has many forms produced in many different situations.

๐–๐ก๐จ ๐ ๐ž๐ญ๐ฌ ๐ข๐ญ?
Any species can get this!

๐–๐ก๐š๐ญ ๐œ๐š๐ฎ๐ฌ๐ž๐ฌ ๐ข๐ญ?
The most common causes of amyloidosis are inflammation and certain types of tumours. In humans, we also see it associated with Alzheimerโ€™s.

Inflammation causes amyloidosis by producing large amounts of ๐€๐€ ๐š๐ฆ๐ฒ๐ฅ๐จ๐ข๐, which is derived from ๐ฌ๐ž๐ซ๐ฎ๐ฆ ๐š๐ฆ๐ฒ๐ฅ๐จ๐ข๐ ๐€. SAA is an ๐š๐œ๐ฎ๐ญ๐ž ๐ฉ๐ก๐š๐ฌ๐ž ๐ฉ๐ซ๐จ๐ญ๐ž๐ข๐ง, which is a group of proteins produced during inflammation. SAA is broken down in to AA amyloid, so when there is chronic inflammation leading to large amounts of SAA, AA amyloid can accumulate.

๐€๐‹ ๐š๐ฆ๐ฒ๐ฅ๐จ๐ข๐ is produced by tumours of ๐ฉ๐ฅ๐š๐ฌ๐ฆ๐š ๐œ๐ž๐ฅ๐ฅ๐ฌ, which are the cells that produce antibodies. Antibodies are made up of ๐ฅ๐ข๐ ๐ก๐ญ ๐œ๐ก๐š๐ข๐ง๐ฌ, so when there is a plasma cell tumour with tons of antibodies produced, these light chains can accumulate in large numbers.

๐–๐ก๐ฒ ๐ข๐ฌ ๐ญ๐ก๐ข๐ฌ ๐š ๐ฉ๐ซ๐จ๐›๐ฅ๐ž๐ฆ?
Amyloid tends to accumulate in the kidney and liver, causing expansion of these tissues by take up space in the organ. Eventually, this can lead to pressure ๐ง๐ž๐œ๐ซ๐จ๐ฌ๐ข๐ฌ (cell death), which often heals by scarring. If enough of the organ is affected, you may see signs of liver failure or kidney failure.

๐‡๐จ๐ฐ ๐ข๐ฌ ๐ข๐ญ ๐๐ข๐š๐ ๐ง๐จ๐ฌ๐ž๐?
Amyloidosis is typically diagnosed on biopsies or at necropsy. The tissue will generally look quite pale and have a waxy texture, due to the accumulation of the protein. A fun magic trick that pathologists might try is applying ๐‹๐ฎ๐ ๐จ๐ฅ’๐ฌ ๐ฌ๐ญ๐š๐ข๐ง to the tissue, which will stain the amyloid dark brown, and make it easy to identify!

On histology, amyloid has more fun colour changing properties. When a ๐‚๐จ๐ง๐ ๐จ ๐ซ๐ž๐ stain is applied to the tissue sample, the tissue will glow ๐š๐ฉ๐ฉ๐ฅ๐ž ๐ ๐ซ๐ž๐ž๐ง under polarized light. Funky!

๐‡๐จ๐ฐ ๐ข๐ฌ ๐ข๐ญ ๐ญ๐ซ๐ž๐š๐ญ๐ž๐?
Unfortunately there is no specific treatment for amyloidosis, other than identifying and treating the underlying cause.

๐๐ก๐จ๐ญ๐จ๐ฌ
1-2) A sample of a kidney and a liver with amyloidosis.
3-4) Kidneys with Lugolโ€™s stain applied!
5-7) What amyloid looks like under our typical stain, H&E, then under Congo Red with normal light, and then under polarized light.
8-9) Another Congo Red and polarized light example!

๐’๐จ๐ฎ๐ซ๐œ๐ž๐ฌ
Maxie, G. Jubb, Kennedy and Palmerโ€™s Pathology of Domestic Animals, Volume 2. Sixth Edition.

Photos 1-4, 8-9 ยฉ Noahโ€™s Arkive contributors King, McGavin, Read, Williams, Crowell licensed under CC BY-SA 4.0.
Photos 5-7ยฉ University of Calgary Diagnostic Services Unit.

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