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22 Cards in this Set
- Front
- Back
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What are the clinical features of amyloid |
Case 1: 45F history of multiple myeloma, gradually developed difficulty speaking, PE disclosed macroglosia = AL, treat the multiple myeloma no good Rx
Case 2: 39F arthritis with IgM autoantibodies to IgG PE showed hepatomegaly = AA
Case 3: 75M autopsy shows thickening of endocardium, amorphous material between myocardial fibers stained with congo red ATTR |
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What is amyloid |
tissue deposits of fibrilar protein, extracellular with mutliple known cases |
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What is the Virchow method of detection |
amyloid turns brown with iodine,
turns blue with H2SO4
affinity for congo red
green yellow birefringence under polarized light
beta sheets |
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What is the structure of the amyloid |
anti parallel beta pleated sheet conformation |
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What are the features of amyloid |
insolube in tissue
accumulates once deposited
results in pressure induced atrophy |
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What is the P component |
not really amyloid but part of the deposit
has pentagonal structure, has been refered to the doughnut portion
binds to amyloid deposits and stabilizes them, non-fibrillar, non congophilic
formed by the liver and circulates as an alpha globulin in serum as precursor molecule SAP |
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What are some of the types of amyloid protein |
Immunoglobulin light chains AL
amyloid associated AA
Transthyretin (prealbumin) ATTR |
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What are some of the stimuli for each type of amyloidosis |
Unknown carcinogen = AL
Chronic inflammation = AA
Mutation = Transthyretin |
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What are the characteristics of AL protein |
so called primary amyloidosis, often the lambda light chains, is systemic
occurs in two forms both of which are from monoclonal populaton |
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What is plasma cell dyscrasia |
AL amyloid caused by multiple myeloma
plasma cell hyperplasia = primary amyloidosis and is more common |
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What is the pathology of AL |
deposits in synovium
cardiac involvement
macroglossia uncommon in other forms
and skin |
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What are the char. of AA protein |
secondary amyloidosis
stimuated by IL-1/6 in liver, SAA acute phase reactant
occurs in two forms 1. chronic inflammation related with osteomyelitis and renal disease 2. familial mediterranean fever |
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In what cases does intestinal amyloidosis occur |
AA and AL |
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What are the properties of familial Mediterranean fever |
recessvie
mutation in MEFV gene on 16p arm codes for pyrin whic is located in polys and controls inflammation
disease presents as intermittant acute atacks 1-3 days
common in sephardics, turks, arab, aermenias skin rash on ankles, renal diease due to deposits |
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What are the characteristics of ATTR protein |
is a normal serum protein that is made in the liver, as well as choroid plexus and retina
transports thyroxine
amyloidosis in two forms 1. familial neuropathies 2. cardiac, senile (no symptoms) and familial cardiomyopathy |
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What are the characteristics of senile cardiac amyloidosis |
amyloid deposits in subendocardial
most often left atrium
seen in elderly |
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What are the properties of familial cardiomyopathy |
usually familial
cardiomegaly
congestive heart failure
extensive myocardial deposits of amyloid |
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What is the role of RNAi in amyloidosis |
siRNAs can be used to target ATT mRNA and lead to lowered levels |
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What is the role of B2 microglobulin |
normal serum protein
elevated in chronic renal disease
amyloid deposits in synovium tendons and carpal ligaments
patients have arthritits or CARPAL TUNNEL syndrome |
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What is endocrine related amyloidosis |
occurs in most endocrine organs most often pancrease in type two diabetes
will replace the islet cells |
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What is the presentation of Beta amyloid |
dementia and alzheimers disease can result from amyloid plagues in the brain |
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How do we Dx amyloidosis |
if systemic as in AL or AA rectal gingival or ab fat
cardiac myocardial bx kidney bx never hepatic |
