amyloidosis + other Flashcards
amyloidosis is the
abnormal aggregation of protein (or their fragments into β-sheat
amyloidosis –> …. (it causes in the cellular level)
damage and apoptosis
amyloidosis - common types
- AL (primary) 2. AA (secondary) 3. dialysis-related
- heritable 5. age related (senile) systemic
- organ specific
primary (AL) amyloidosis - mechanism
deposition of proteins from Ig Light chains (amyloid Light: AL)
primary (AL) amyloidosis is associated with
plasma cell dyscrasias (multiple myeloma)
primary (AL) amyloidosis often affects …(including, and causes)
multiple organs including renal (nephrotic syndrome), cardiac (restrictive cardiomyopathy, arrhythmia), hematologic, (easy bruising, splenomegaly), GI (hepatomegaly), neurologic (neuropathy)
secondary (AA) amyloidosis - seen with
chronic inflammatory conditions such as RA, IBD, spondyloarthropathy, protracted infection, famlilial Mediterranean fever
secondary (AA) amyloidosis - fibrils composed of
serum Amyloid A
secondary (AA) amyloidosis - affects
often multisystem like AL amyloidosis
dialysis related amylodisis - fibrils composed of
β2-microglobulin
dialysis-related amylodisis - seen in
patients with end-stage-renal-disease and/or long-term dialysis
dialysis-related amylodisis may present as
Carpal tunnel syndrome
heritable amyloidosis (including….)?
heterogenous group of disorders, including familial amyloid polyneuropathies due to transthyretin gene mutation
heritable amyloidosis is a heterogenous group of disorders, including (and due to..)
familial amyloid polyneuropathies due to transthyretin gene mutation
familial amyloid polyneuropathies are due to
transthyretin gene mutation
age-related (senile) systemic is due to
deposition of normal (wild-type) transthyretin in myocardium and other sites
age-related (senile) systemic vs primary amyloidosis according to cardiac function
cardiac dysfunction in age-related (senile) systemic has a slower progression relative to primary amyloidosis
organ specific amyloidosis?
amyloid deposition localized to a single organ
organ specific amyloidosis - Most important form is
Alzheimer disease
Alzheimer disease - mechanism of amyloidosis
deposition of β-amyloid protein cleaved from amyloid precirsor protein (APP)
islet amyloid polypeptid is commonly seen in …. (and is caused by)
diabetes mellitus type 2
deposition of amylin in pancreatic islets
Amylin is a
peptide hormone that is cosecreted with insulin from the pancreatic beta-cell
amyloidosis - appearance
- congo red stain
- congo red stain shows apple green birefringence under polarized light
- H% eosin