Amyloidosis Flashcards
2 types of amyloid
AL: from light chain
AA: from chronic inflammation (from SAA)
Amyloid is what type of protein and deposits where
misfolded protein
deposits in extracellular spaces around blood vessels
1˚amyloidosis
amyloid type
disease association
AL amyloid from light chain
multiple myeloma association
2˚ amyloidosis
amyloid type
disease association
AA amyloid from SAA amyloid
due to chronic inflammatory conditions or malignancy, or familial mediteranian fever
Familial Mediterranean Fever
inheritance
pathology
presentation
recessive
dysfunction of neutrophils (SAA–>AA amyloidosis)
fever, acute serosal inflammation
(heart, peritoneum, others)
Most commonly affected organ in amyloidosis
kidney
Heart in amyloidosis
restrictive cardiomyopathy
Tx of organs affected by amyloidosis
need transplant
amyloid cannot be removed
Senile cardiac amyloidosis
non-mutated serum transthyretin deposits in heart
usually asymptomatic
Familial amyloid cardiomyopathy
recessive
mutated serum transthyretin deposits in heart
=> restrictive cardiomyopathy
Amyloid in type 2 diabetes mellitus
overproduction of insulin early in disease
amylin (derived from insulin) is produced due to this
amylin deposits around islets
destruction of islets
now you need exogenous insulin
Alzheimer results from deposition of this amyloid
Aβ amyloid from β-amyloid precursor protein
Population at risk of early onset Alzheimers
Down syndrome
β-APP (β-amyloid precursor protein) found on chromosome 21
mutated β-APP leads to faster deposition
Dialysis-associated amyloidosis
etiology
β2 microglobulin deposits in joints
normally stabilized MHC-1
but cannot be easily filtered in dialysis membrane
Medullary carcinoma of thyroid
amyloid etiology
calcitonin deposits from increased release of calcitonin
“tumor cells in amyloid background)
