Amyloidosis
Amyloidosis is a rare and serious disease that causes abnormal proteins to build up in the body's tissues.[1][2] These abnormal, insoluble proteins are called amyloid fibrils.[1] Amyloidosis can be fatal.[2]
In "local amyloidosis," amyloid deposits build up in just one part of the body, or in a single organ system. In "systemic amyloidosis," the deposits build up in organs and tissues throughout the body.
Because amyloidosis can affect different parts of the body, it can cause many different symptoms. It can be challenging to confirm that amyloid fibrils are in the body's tissues, which can delay diagnosis.
Types
There are many different types of amyloidosis. Each is caused by the formation of amyloid fibrils. However, these fibrils develop from different types of proteins in different patients.[2][3] About 30 different proteins are known to form amyloid fibrils in humans.[4] Amyloidosis is named and classified according to which protein is causing a person's amyloid fibrils.[3]
Though this disease is rare, many famous people have died from amyloidosis, including Martin McGuinness, David Lange, Robert Jordan Robert P. Casey and Pervez Musharraf.
Amyloidosis Media
Skin features of amyloidosis cutis dyschromica. Hyperpigmented and hypopigmented macules on (A) lower legs, (B) back and waist, (C) waist. (D) Individual blisters on upper arm.
Small bowel duodenum with amyloid deposition Congo red 10X
Amyloidosis, dystrophic calcification
Small bowel duodenum with amyloid deposition 20X
Amyloidosis, Node, Congo Red
Amyloidosis, blood vessels, H&E
Amyloidosis, lymph node, H&E
Amyloidosis, lymph node, polarizer
Micrograph showing amyloid deposition (red fluffy material) in the heart (cardiac amyloidosis). Congo red stain.
References
- ↑ 1.0 1.1 Wechalekar, Ashutosh D.. Systemic amyloidosis. Lancet (London, England) 387 (10038) (2015-12-21). p. 2641–2654. doi:10.1016/S0140-6736(15)01274-X.
- ↑ 2.0 2.1 2.2 Pepys, Mark B.. Amyloidosis. Annual Review of Medicine 57 (2006-01-01). p. 223–241. doi:10.1146/annurev.med.57.121304.131243.
- ↑ 3.0 3.1 Oxford Textbook of Medicine (2010)Oxford University Press. ISBN 9780199204854. doi:10.1093/med/9780199204854.001.1.
- ↑ Sipe, Jean D.. Nomenclature 2014: Amyloid fibril proteins and clinical classification of the amyloidosis. Amyloid 21 (4) (2014-12-01). p. 221–224. doi:10.3109/13506129.2014.964858.
Other websites
- National Press Club Luncheon with Michael York, August 2016. Michael York who has amyloidosis, speaks from 4:00-60:00
