7. Amyloidosis Flashcards
What is amyloid?
Misfolded protein deposits between cells which interrupt organs
How does amyloid appear on H&E?
Pink blobs with no nucleus
What is the composition of amyloid?
Non-branching fibrils (beta pleated sheet)
Nonfibrillar pentagonal glycoprotein
Proteoglycans
What other stain and technique is used to diagnose amyloidosis?
Green birefringence under polarised light when stained with Congo red
What is AL amyloidosis?
Amyloid light chain
Over production of immunoglobulin light chains
What disease is AL amyloidosis associated with?
Myeloma
How could AL amyloidosis be detected on serum electrophoresis?
M protein spike
What organs are commonly affected by AL amyloidosis?
Kidneys
Heart
What is the treatment for AL amyloidosis?
Stem cell transplant
Chemo
What is AA amyloidosis?
Liver produces SAA which is an acute phase response protein in response to chronic inflammation
This becomes AA amyloid
What diseases is AA amyloidosis associated with?
RA
Chronic infections
Neoplasms
What clinical consequences are associated with AA amyloidosis?
Renal disease Infection Heart failure Bowel perforation GI bleeding
How is AA amyloidosis treated?
By treating underlying inflammation
What age range is amyloidosis most common in?
50-60
How might the kidneys present with amyloidosis?
Proteinuria
Nephrotic syndrome
Renal insufficiency
How might the heart present with amyloidosis?
Heart failure
Restrictive cardiomyopathy
How might the liver present with amyloidosis?
High LFTs
Hepatomegaly
How might the GIT present with amyloidosis?
Malabsorption
How might the spleen present with amyloidosis?
Dysfunction
Abnormal RBCs
What do organs with amyloidosis look like?
Enlarged
Pale and grey
‘Waxy’
How might the tongue present amyloidosis?
Macroglossia
How might synovial tissue present amyloidosis?
Carpal tunnel syndrome
What are 2 examples of localised amyloidosis?
Endocrine related
Intercerebral
