Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
22 Cards in this Set
- Front
- Back
|
Amyloidosis is a __________ deposited between cells in various tissues and organs of the body in a wide variety of clinical settings. |
proteinacious substance. |
|
|
Matt in 10 years? |
|
|
|
Amyloid fibrils are generally what size? |
7.5-10 nm |
|
|
Structure and characteristics of amyloid protein (5)? |
non-branching beta-pleated sheets 7.5-10 nm in diameter
insoluble resistant to proteolysis stain with congo red apple green bifringence under polarized light |
|
|
Chemical nature of amyloid (3)? |
protein fibrils "P" component proteoglycans- GAGs |
|
|
P-component is made up of? |
alpha-1 glycoprotein, structural homology to C reactive protein
looks like a "stack of coins" and is present on all forms of amyloid |
|
|
Major proteinaceous components of amyloid (3)? |
immunoglobulin light chains, variable regions of kappa and lambda- AL fibril
acute phase protein produced in the liver in inflammatory conditions- AA fibril
transthyretin transports thyroxine and retinol, familial associated and senile amyloidosis- AF fibril |
|
|
Minor proteinaceous components of amyloid? |
beta-2 microglobulin beta-2 amyloid protein hormones- procalcitonin, amylin keratin |
|
|
formation of AL amyloid? |
misfolded and undigested light chain produced by plasma cells, binds proteoglycans and GAGs in tissues |
|
|
SAA (AA fibril) behaves as a ________ and is produced in the _____. |
acute phase reactant, liver |
|
|
Ways to classify amyloidosis (3)? |
by tissue distribution by major fibril protein if primary, secondary, or inherited
|
|
|
Primary amyloidosis is? |
without detectable cause associated with b-cell malignancies
Ig type protein
|
|
|
Secondary amyloidosis is associated with? |
chronic inflammation and tissue damage |
|
|
AL is associated with which amyloid disease? Clinical presentation? |
plasma cell dyscrasia, Ig fibrils deposited in various organs leading to dysfunction
associated with multiple myeloma
Clinical: age 65-70 fatigue, wt. loss CHF symptoms, nephrotic syndrome |
|
|
AA is associated with what abnormality? |
inflammation and tumors |
|
|
AF is associated with what abnormality? |
FAP, Cardiac, senile amyloidosis |
|
|
Amyloid deposit in the kidneys leads to what defect? Early lesions? Late lesions? |
Early: deposits in the podocytes and destruction of basement membrane in bowman's capsule
Late: obliteration of capillary lumen and glomeruli
major cause of mortality |
|
|
Diagnosis of amyloidosis requires? |
tissue biopsy:
adipose gingival/rectal biopsies visceral organ biopsies serum/electrolytes/kumquat jbone marrow |
|
|
_____ spleen includes deposits in splenic white pulp, with gross appearance of numerous small white spots.
______ spleen includes extensive involvement of red pulp and ultimately white pulp. |
sago spleen; lardaceous |
|
|
Early lesions in the Liver occur in?
Late lesions? What is unusual about live function during these? |
early: deposits in space of disse late: total replacement of parenchyma
liver function is normal despite severe involvment |
|
|
Treatment options for amyloid? |
Mephalan with or without prednisone or dexamethasone
not very effective with median survival of ~16-18 months
intravenous melphalan w/ autologous stem cell transplant median survival 73 mo |
|
|
Amyloidosis is not one disease but a _______ that have similar ______ deposits with similar morphologic, structural and staining properties despite fundamentally different amino acid sequences, _______, and ______. |
group of diseases, protein; clinical setting, prognosis |
