Clinical sesh - amyloids 09/19 Wasan Flashcards
primary amyloidosis (AL)
abnormal and excessive production of antibodies by plasma cells results in amyloid plaque build-up and, in 50% of patients, cardiac involvement
how many different proteins are know to be able to form amyloid fibrils
over 25
T/F amyloid fibrils formed by different proteins are markedly different
false - structure and properties of all amyloid fibrils are remarkably similar
secondary amyloidosis (AA)
deposition of serum amyloid A protein (an inflammatory protein); associated with chronic inflammatory disorders like rheumatoid arthritis (rarely cardiac involvement)
describe some clinical manifestations of primary amyloidosis (AL)
kidney involvement
peripheral neuropathy
carpal tunnel
hepatomegaly (enlarged liver, elevated enzymes)
macroglossia (amyloid deposits in skeletal muscle)
cardiac involvement
bleeding diathesis (deposits in blood vessels, liver, spleen)
purpura - purple skin from subcu bleeding
what is the gold standard for diagnosing amyloidosis
tissue biopsy
T/F amyloidosis is a multisystem disease
true - can affect circulatory system, GI system, urinary system, nervous system, etc
what is the primary aim of treating amyloidosis?
reducing abundance of precursor protein forming amyloid fibrils
what is the appearance of amyloid fibrils in congo red dye under non-polarized light?
they look kind of like pink dense irregular connective tissue where it shouldn’t be – e.g. between fascicles of cardiac muscle
what is the appearance of amyloid fibrils in congo red dye under polarized light?
appears as an apple green accumulation between normal tissues/cells
what kind of protein misfolding disease is likely to occur with multiple myeloma / plasma cell dyscrasia?
primary amyloidosis (AL) abnormal and excessive production of antibodies by plasma cells results in amyloid plaque build-up and, in 50% of patients, cardiac involvement
