Amyloidosis Flashcards
1
Q
What are the various stains that can be used for amyloid proteins? What colours do they give?
A
H&E - pink, hyaline
Methyl violet/crystal violet - metachromasia (rose -pink)
Congo red - Pink-red (under light microscopy); red-green birefringence (under polarising light)
Thioflavin-T/Thioflavin-S - fluorescent under UV light
Immunohistochemistry - amyloid protein reacts with antibody
2
Q
What is the difference between AA and AL proteins?
A
Both are amyloid proteins, however-
- AL is made of light chain Igs in plasma cells
- AL is non-Ig derived but is synthesised in liver, in responses to secretion of IL-1, IL-6
3
Q
What is transthryetin?
A
- normal serum protein (transports retinol and thyroxine)
- mutated foms typical of familial congenital disease
- normal form found in hearts of aged patients (senile cardiac amyloidosis)
4
Q
What disease cause secondary amylodosis?
A
(Previously)
- bronchiecstasis
- TB
- osteomyelitis
(Nowadays)
- rheumatoid arthritis
- ankylosing spondylosis
- inflammatory bowel diseases
- chronic skin infections (drug abuse)
- solid tumours
5
Q
What are the features of famililal mediterranean fever?
A
- autosomal recessive
- systemic amyloidosis (widespread)
- xs IL-1 synthesis
- fever attacks + serous inflammation
- AA proteins
6
Q
What are the features of familial idiopathic neuropathy?
A
- autosomal dominant
- amyloid deposits in peripheral and autonomic nerves
- made of mutant transthyretin
7
Q
What are the features of familial idiopathic neuropathy?
A
- autosomal dominant
- amyloid deposits in peripheral and autonomic nerves
- made of mutant transthyretin
