Amyloidosis Flashcards
What is Amyloid
Pathological proteinaceous substance deposited in the extra cellular space
Systemic disease abnormal protein folding
How is the differentiation of Amyloids
Congo red stain
How many biochemical forms does Amyloid have?
3 major
Several minor
How it he physiologic nature of the amyloid
Continuous non branching fibrils
How is the chemical nature of the amyloid
Fibril proteins
P component
Glycoproteins
Classification of amyloidosis
Primary= AL
Reactive Systemi (Secondary)= AA
What is AL
Amyloid Light Chain
Derived from Ig light chains
Produced in plasma cells
Primary
AL derived from
Ig light chains produced in plasma cells
Which Amyloid type is primary
AL
Abeta amyloid produced from
Beta amyloid precursor protein
ABETA amyloid found in
The cerebral lesions of Alzheimer’s disease
Which amyloid is the secondary
AA
Which amyloid does not have structural homology to Igs
AA
AA fibrils derived by
Proteolysis from a precursor in the serum called Serum Amyloid Associated which is synthesized in the liver and circulates in association with Heavy Dense Lipoprotein
SAA is increased in
Inflammatory states as part of the acute phase response so it is associated with chronic inflammation
What is Transthyretinin protein
Anormal protein
Binds and transports thyroxine and retinol
Have mutations
What is Senile Systemic Amyloidosis
Caused by accumulation of Transthyretin
Affects heart and tendons of elderly people
Beta microglobulin
Component of MHC class 1 molecules
Accumulates in long term hemodialysis
Primary and secondary amyloids are associated with which diseases
Primary = ımmunocyte disorder, plasma cell dyscraia , myeloma
Secondary= complication of an underlying chronic inflammatory or tissue destructive process
Reactive systemic amyloidosis
Amyloid deposits in this pattern are systemic distribution and are composed of AA protein associated with inflammatory conditions as TBC,bronchiectasis,chronic osteomyelitis or CT disorders, inflammatory bowel disease
Hemodialysis associated amyloidosis
Heredofamilial amyloidosis
Endocrine amyloid
Amyloid of aging
Which organ amyloidosis is the most common and potentially the most serious form of organ involvement
Kidney
2 forms of Spleen deposits
1- Sago Spleen : splenic follicles producing tapioca like granules
2- Lardeceous Spleen : the walls of the spleen sinuses and CT framework
Accumulation of Liver
First: Disse spaces
Then: hepatic parenchyma cells
Sinusoids causes pressure atrophy
