Term
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Definition
| heterogeneous group of disorders characterize by the accumulation of diverse extracellular proteins |
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Term
| What does amyloidosis look like in an H&E stain? |
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Definition
| smooth, glassy, eosinophilic, homogeneous, extracellular material |
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Term
| What does amyloidosis look like in a congo red stain in light microscopy? |
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Definition
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Term
| What does amyloidosis look like in a congo red stain in polarization microscopy? |
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Definition
| apple green-yellow birefringence |
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Term
| What is the structure of amyloidosis as seen my transmission electron microscopy? |
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Definition
| straight, criss-crossing fibrils |
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Term
| What is the conformation of amyloidosis as seen in x-ray crystallography? |
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Definition
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Term
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Definition
overproduction of normal or synthesis of abnormal protein => protein misfolding
if there's insufficent degradation => aggregation into insoluble fibrils & accumulation in tissue |
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Term
| *What is amyloid composed of? |
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Definition
primarily fibrillary proteins
sm. contribution of glycoproteins
serum amyloid P component |
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Term
| *How many biochemical types are there of amyloid? |
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Definition
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Term
| What are the 3 major biochemical types of amyloid? |
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Definition
1) AL (amyloid light chain)
2) AA (amyloid-associated)
3) Aβ (β-amyloid) |
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Term
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Definition
| complete or partial Ig light chains, usually λ |
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Term
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Definition
| serum amyloid-associated (SAA) protein |
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Term
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Definition
| an acute phase reactant synthesized by hepatocytes |
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Term
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Definition
| cleaved fragment of amyloid precursor protein (APP) |
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Term
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Definition
| a neuronal cell surface molecule |
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Term
| What are the 4 minor biochemical types of amyloid? |
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Definition
| 1) ATTR (transthyretin amyloid)
2) Aβ2M (β2-microglobulin amyloid)
3) AE (endocrine amyloid)
4) PrP amyloid (prion protein amyloid) |
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Term
| What is ATTR comprised of? |
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Definition
| normal-sequence or variant-sequence transthyretin |
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Term
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Definition
| serum protein tht binds & transports thyroxine & retinol |
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Term
| What is Aβ2M composed of? |
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Definition
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Term
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Definition
| protein in the serum & on the surface of most nucleated cells as a component of MHC I |
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Term
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Definition
| protein hormones s.a. amylin, atrial natriuretic factor, calcitonin & insulin |
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Term
| What are the 4 types of systemic amyloidosis? |
|
Definition
1) *Primary
2) Secondary/Reactive
3) Hemodialysis-associated
4) Senile systemic |
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Term
| *What causes primary amyloidosis? |
|
Definition
plasma cell dyscrasia => overproduction of a monoclonal Ig/free light chains => protein misfolding
if there's insufficient degradation => amyloid accumulation |
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Term
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Definition
| an abnormal bodily condition |
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Term
| What biochemical type of amyloid is associated with primary amyloidosis? |
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Definition
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Term
| What causes secondary/reactive amyloidosis? |
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Definition
chronic inflammation => long-standing hepatic production of serum amyloid-associated protein (SAA) => protein misfolding
if there's insufficient degradation => amyloid accumulation |
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Term
| What amyloid biochemical type is associated with secondary/reactive amyloidosis? |
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Definition
|
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Term
| What causes hemodialysis-associated amyloidosis? |
|
Definition
| long term hemodialysis => retention β2-microglobulin in serum => protein misfolding
if there's insufficient degradation => amyloid accumulation |
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Term
| What biochemical type of amyloid is associated with hemodialysis-associated amyloidosis? |
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Definition
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Term
| What causes senile systemic amyloidosis? |
|
Definition
accumulation of normal-sequence transthyretin in elderly individuals
can be due to a genetic mutation resulting in abnormal transthyretin molecules that are amyloidogenic |
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Term
| What biochemical type of amyloid is associated with senile systemic amyloidosis? |
|
Definition
|
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Term
| What organ is primarily affected by senile systemic amyloidosis? |
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Definition
|
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Term
| What are the 3 types of localized amyloidosis? |
|
Definition
1) Cerebral
2) Endocrine
3) Other |
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Term
| When is cerebral amyloidosis seen? |
|
Definition
| with Alzheimer's & cerebral amyloid angiopathy |
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Term
| What biochemical type of amyloid is associated with cerebral amyloidosis? |
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Definition
|
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Term
| What are 3 forms of endocrine anyloidosis? |
|
Definition
1) Medullary Thyroid Carcinoma
2) Type 2 DM
3) Isolated atrial amyloidosis |
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Term
| What biochemical type of amyloid is associated with endocrine amyloidosis? |
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Definition
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Term
def
Medullary thyroid carcinoma |
|
Definition
| malignancy of C cells (parathyroid cells) |
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Term
| Where are associated amyloid deposits derived from in medullary thyroid carcinoma? |
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Definition
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Term
| Where is there amyloid accumulation in Type 2 DM? |
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Definition
| in the pancreatic iselts of Langerhans |
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Term
| Where are amyloid deposits derived from in isolated atrial amyloidosis? |
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Definition
| atrial natriuretic factor |
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Term
| What can cause Other localized amyloidosis? |
|
Definition
| nodular deposition of amyloid in a wide variety of organs |
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Term
| What are the 2 herediary/familial amyloidosis? |
|
Definition
1) Familiar Mediterranean Fever
2) Familial Amyloidotic Neuropathies |
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Term
| How common are hereditary/familial amyloidosis conditions? |
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Definition
|
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Term
| What causes familial mediterranean fever? |
|
Definition
| autosomal recessive disorder of pyrin => systemic accumulation of amyloid |
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Term
| What biochemical type of amyloid is associated with familial mediterranean fever? |
|
Definition
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Term
Sx
familial mediterranean fever |
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Definition
recurrent fever
abdominal & joint pain
serositis (inflammation of serous tissue)
overproduction of IL-1 (chronic inflammation) |
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Term
| What causes familial amyloidotic neuropathies? |
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Definition
| autosomal dominant disorder of transthyretin => deposition of amyloid (esp. in peripheral & autonomic nerves) |
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Term
| What biochemical type of amyloid is associated with familial amyloidotic neuropathies? |
|
Definition
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Term
| What do is the gross characteristic s of organs with amyloidosis in general? |
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Definition
|
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Term
|
Definition
| initially nonspecific => depends on site & extent of organ involvement |
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Term
| *Where does amyloid accumulate in kidney? |
|
Definition
| glomeruli, ateries, & interstitium |
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Term
| *What happens due to amyloidosis in the kidney? |
|
Definition
| proteinuria & eventual renal failure |
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Term
| Where does amyloid accumulate in the lover? |
|
Definition
| initially within the space of Disse => widespread involvement |
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Term
| What leads to widespread amyloid accumulation in the liver? |
|
Definition
| pressure atrophy of hepatocytes |
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Term
| What happens to liver function in amyloidosis? |
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Definition
|
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Term
| Where does amyloid accumulate in the spleen? |
|
Definition
| Either in the white pulp or red pulp |
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Term
|
Definition
| white pulp accumulation of amyloid |
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Term
|
Definition
| red pulp accumulation of amyloid |
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Term
| What happens due to amyloidosis in the spleen? |
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Definition
|
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Term
| What do subendocardial amyloid deposits cause in the heart? |
|
Definition
| conduction disturbances & arrythmias |
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Term
| What do myocardial amyloid deposits cause in the heart? |
|
Definition
| pressure atrophy of cardiac myocytes & restrictive cardiomyopathy => congestive heart failure |
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Term
| What does amyloid deposition in endocrine organs cause? |
|
Definition
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Term
| What does amyloid accumulation in the tongue cause? |
|
Definition
| macroglossia => speech & swallowing impairment |
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Term
|
Definition
| hypertrophy of the tongue |
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Term
| Where in the skin is especially prone to amyloid deposits? |
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Definition
|
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Term
| What happens due to amyloid deposits in the GI tract? |
|
Definition
| difficulties with digestion or absorption |
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|
Term
| Where in the RT can amyloid accumulation occur? |
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Definition
|
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Term
| Which amyloid biochemical type is most frequently associated with joing & tendon amyloidosis? |
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Definition
|
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Term
| What does amyloidosis in joints & tendons cause? |
|
Definition
| carpal tunnel & arthropathy (arthritis) |
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Term
| What amyloid biochemical type is found in blood vessels/senile plaques in the brain? |
|
Definition
|
|
Term
| When are amyloid deposits seen in peripheral & autonomic nerves? |
|
Definition
| Familial amyloidotic neuropathies |
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