Term
| What 2 components comprise AMYLOID |
|
Definition
1. amyloid P 2. fibrillogenic protein (disease-specific) |
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|
Term
how do amyloids look histologically |
|
Definition
amorphous acellular eosinophilic exctracellular |
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Term
| How does amyloid look with Congo red stain |
|
Definition
|
|
Term
| How does amyloid look with polarized light |
|
Definition
| apple green birefringence |
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|
Term
| What stain causes amyloid autofluorescence |
|
Definition
|
|
Term
| What is the structure of amyloid ? |
|
Definition
fibrillar groups of fibers in parallel arrays oriented in different directions Beta pleated sheet formation |
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|
Term
| What requirements must be met for amyloid formation |
|
Definition
1. adequate amount of protein 2. suitable location for deposition 3. altered microenvironment 4. proteolysis of precursor molecule |
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|
Term
| where is amyloid initially deposited? |
|
Definition
| in the vicinity of the endothelial basement membranes |
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|
Term
| What is the negative effect of amyloid deposition |
|
Definition
"cell strangulation" b/c amyloid deposited between cell and their blood supply |
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|
Term
| What would be the appearance of an organ that has amyloid deposits |
|
Definition
organ enlarged organ pale and firm (b/c amyloid is avascular) |
|
|
Term
| Definite primary amyloidosis |
|
Definition
where symptoms tend to occur PRIOR TO diagnosis of underlying plasma cell disorder |
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|
Term
| What is secondary amyloidosis |
|
Definition
| develops in association with PREVIOUSLY DIAGNOSES persistent inflammatory disorder |
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|
Term
| rheumatoid arthritis would be an example of _________amyloidosis |
|
Definition
|
|
Term
| Name two types of familial amyloidosis |
|
Definition
familial mediterranean fever familial amyloiditic polyneuropathy |
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|
Term
| Neutrophil dysfunction, recurrent infections and secondary amyloidosis are characteristic of ______________ |
|
Definition
| familial mediterranean fever |
|
|
Term
| how does familial amyloiditic polyneuropathy occur |
|
Definition
| point mutation in gene for transthyretin |
|
|
Term
| What is responsible for alzheimer's |
|
Definition
|
|
Term
| Which protein is associated with Down's syndrome |
|
Definition
amyloid Beta protein precursor (this is why down's syndrome ppl have high incidence of developing Alzheimer's) |
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|
Term
| islet amyloid polypeptide (amyloid) overproduction is associated with what disease |
|
Definition
|
|
Term
| transthyretin is associated with which type of amyloidosis |
|
Definition
| cardiac senile amyloidosis |
|
|
Term
| AL amyloid is associated with which 2 disorders |
|
Definition
plasma cell disorders B-cell lymphomas |
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|
Term
| A-Beta-2-M amyloid associated with |
|
Definition
|
|
Term
| Creutfeldt-Jakob disease is associated with which type of amyloidosis? |
|
Definition
|
|
Term
| ACAl is amyloidosis is associated with? |
|
Definition
medullar carcinoma of thyroid (thyroid C cells that secrete calcitonin) |
|
|
Term
| What are the clinical maniestation of amyloidosis in heart? |
|
Definition
infiltrative cardiomyopathy --> cardiomegaly heart failure arrythmias |
|
|
Term
| What is the clinical manifestation of amyloidosis in the kidney? |
|
Definition
|
|
Term
| What is the classic feature of amyloidosis |
|
Definition
|
|
Term
How would amyloidosis affect peripheral nerves? |
|
Definition
paresthesias loss of sensation |
|
|
Term
| Why are coagulopathies a feature of amyloidosis |
|
Definition
selective absorption of Factor X by amyloid protein |
|
|
Term
is AL amyloid primary or secondary amyloidosis What about AA? |
|
Definition
AL --> primary AA--> secondary |
|
|
Term
| senile cardiac amyloidosis has what type of amyloid |
|
Definition
|
|
Term
| type 2 diabeted has what type of amyloid ? |
|
Definition
amylin (islet amyloid polypeptide) |
|
|
Term
| plasma cell and B-cell lymphomas are associated with what type of amyloid |
|
Definition
|
|
Term
| medullary carcinoma of the thyroid is associated with what amyloid |
|
Definition
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