Amyloidosis

Question 1

What is amyloidosis?

Answer 1

A group of disorders characterised by extracellular deposition of an insoluble fibrillar protein called amyloid

Question 2

Why does amyloidosis occur?

Answer 2

Acquired or hereditary

Question 3

Clinical features of amyloidosis?

Answer 3

The result of the amyloid deposits affecting the normal structure and function of the affected tissue

Question 4

Investigations of amyloidosis?

Answer 4

Biopsy of affected areas:

Congo red staining combined with polarised light, makes the amyloid proteins appear green

Scintigraphy: use radioactively labelled isotopes, which are taken up by the amyloid and light up on a scan

Question 5

Apart from amyloidosis, what other pathologies is amyloid implicated in?

Answer 5

Alzheimer’s disease, you get amyloid plaques in the brain

Question 6

Management of amyloidosis?

Answer 6

No treatment that can get rid of already deposited amyloid

But you can prevent further release, using chemotherapy to damage the abnormal bone marrow cells and inhibit production of the abnormal proteins.

Treatment of symptoms or organ failure