Amyloidosis

Amyloidosis

definition
deposition of amyloid in the Interstitial tissues +wall of BVs
Structure
Chemical
1. AA
2. AL
3. Transthyretin
4. B2 microglubulin
5. b amyloid protein
physical
structure
95%=non branching fibrills 5%=p components +glycoprotein
non branching fibrills in the shape of crossed B pleated sheets
plasma protein primary amyloidosis light chain Igs
no Igs liver 2ry amyloidosis
normal serum protein transports thyroxin and retinol senile systemic amyloidosis +familial amyloid polyneropathy
long term dialysis synovium+joints+tendon sheath.
alzheimer
Classification
primary
secondary
Myeloma pts serum has Bence Jones prts(kappa ,lambda)
all the other diseases
pathogenesis: defective degradation in bence jones prts
pathogenesis: liver produces SAA under the effect of IL1 which leads to insoluble SAA
site
localized in a single organ
systematic in multiple organs
clinical feature in different organs :
generally : weakness ,weight loss, fatigue
heart: cardiomyopathy+arrhythmia
in kidney: renal failure
in liver:hepatosplenomegaly
diagnosis: must be done by biopsy stained by Congo red/in generalizes most cases have rectal and gingival amyloid.
sites: heart,skin,peripheral nerves,tongue,respiratory
sites:kidney,liver,spleen, LNs,adrenal,thyroid.

Resource summary

	Created by Lamees Sheikh about 4 years ago