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22 Cards in this Set
- Front
- Back
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where would you find the fibrillar proteins in a patient with amyloidosis?
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extra-cellular pink, glassy deposits in SUBENDOTHELIAL locations in or around blood vessels
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What stain/process would you use to detect amyloidosis?
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Congo Red
-brick red on regular light Polarized Light -apple green birefringence Thioflavin T -fluoresces with UV light Specific antibodies -to different fibrillar proteins |
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What are the 5 different components of amyloidosis
"PAGE" |
fibrillary PROTEIN POOL
Amyloid P component Glycosaminoglycan apoE |
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proteins in amyloid deposits have what type of secondary structure?
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beta-pleated sheets
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pentagonal donut shaped protein found in NORMAL BASEMENT MEMBRANES and all types of AMYLOID
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amyloid P component
derived from SAP (Serum Amyloid P component) |
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What are the components of BM that are found in amyloidosis?
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SAP (serum amyloid P component)
Laminin Collagen IV Glycosaminoglycan (GAG) --Perlecan --Heparan Sulfate Proteoglycan |
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what type of amyloidosis is composed of Ig light chains either Lambda (dominant) or Kappa?
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primary amyloidosis (AL type)
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True or False
the majority of Primary Amyloidosis are associated with overt B-cell or Plasma cell Dyscrasias (myeloma). |
FALSE
primary amyloidosis is NOT associated with it |
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what type of amyloidosis is associated with B-cell malignancy
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Secondary Type of AL AMYLOIDOSIS
primary is NOT associated with B-cell malignancy |
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what amyloidosis is associated with chronic inflammatory diseases?
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AA type or Secondary Amyloidosis
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liver protein is AA (amyloid associated)
--precursor is an ? |
Acute Phase Reactant Protein
SAA (serum amyloid associated) |
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Deposition of mutated proteins such as TRANSTHYRETIN, apolipoprotein, lysozyme, etc.
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Hereditary or Familial Amyloidoses
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? causes midlife onset of Hereditary or Familial Amyloidoses.
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Mutated ATTR (Amyloid Tran-Thy-Retin)
--aka TRANSTHYRETIN --transport protein for thyroid hormone and retinol |
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in HEMODIALYSIS ASSOCIATED AMYLOIDOSIS
? is not filtered by dialysis and amyloid is deposited in soft tissue of joints and tendons |
β2 - microglobulin
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what are the 2 classical findings of LOCALIZED AMYLOIDOSES in Alzheimer's Disease?
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Neuritic Plaques
-amyloid surrounded by dystrophic neurites Neurofibillary Tangles -intra-neuronal deposition of double helices of hyperphosphorylated TAU potein |
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what are the classical finds of LOCALIZED AMYLOIDOSES in Senile Cardiac Amyloidosis?
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sudden accumulation of normal Transthyretin in elderly heart for unknown reasons
--PROGRESSIVE HEART FAILURE |
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What type of amyloidoses is primarily SYSTEMIC and has the widest spectrum of organ involvement
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AL type
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Macroglossia is ONLY seen in what type of amyloidosis?
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AL type
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Hepatosplenomegaly is seen more pronounced in ? and less in ? type of amyloidoses
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pronounced in AA type
less in AL type |
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What type of coagulation deficiency will most likely occur in amyloidosis?
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Factor X deficiency
more likely in primary amyloidosis |
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? type presents as renal disease with hepatomegaly and/or splenomealy BUT cardiac failure is rare
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AA present
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? type presents with Neuropathy (common) and Cardiac arrhythmias from conduction infiltration
LESS renal involvement!! |
ATTR (Met 30)
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