Amyloidosis Flashcards
What is amyloidosis?
group of diseases characterized by deposition of an extracellular protein that has specific properties
What is primary amyloidosis?
had amyloid light chain (AL) amyloidk, whose fibrillary protein is made of kappa or lambda light chains
What disorders may primary amyloidosis be seen in?
plasma cell disorders (mult. myeloma, B -cell lymphomas(
What is reactive systemic amyloidosis?
(secondary amyloidosis)
has amyloid-associated protein, whose precursor is serum amyloid A (SAA), an acute phase reactant produced by the liver which is elevated with oncogin chronic inflammation and neoplasia
When can reactive systemic amyloidosis be seen?
in a wide variety of chronic diseases, including rheumatoid arthritis, SLE, TB, bronchiectasis, osteomyelitis, IBD and cancer
What is familial mediterranean fever? What is it characterized by?
AA type amyloid with fibrillary protein composed of serum amyloid A
recurrent inflammation, fever, and neutrophil dysfunction
Hemodialysis-associated amyloidosis? Characteristics?
AB2M type amyloid with precursor proteibn B2-microglobulin
may cause carpal tunnel syndrome and joint disease
Senile cerebral amyloidosis? What conditions is it seen in?
(Alzheimer disease)
Has AB type amyloid with fibrillary protein composed of B-amyloid precursor protein (BAPP) found in Alzheimer plaques
Senile cardiac/systemic amyloidosis? Complications?
hasATTR type amyloid with fibrillary protein composed of transthyretin.
seen in men older than 70 and may cause heart failure as a result of restrictive cardiomyopathy
Endocrine type amyloidosis? Complications?
seen in medullary carcinoma of the thyroid (procalcitonin), adult onset diabetes (amyline), and pancreatic islet cll tumors (amylin)
What is hte most commonly involved organ in the systemic form of amyloidosis?
the kidney
How is AL amyloidosis diagnosed?
by serum and urinary protein electrophoresis and immunoelectrophoresis
