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Pathobio Exam 1
Set 5: Amyloidosis
72
Pathology
Graduate
10/07/2011

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Term
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Amyloidosis
Definition
heterogeneous group of disorders characterize by the accumulation of diverse extracellular proteins
Term
What does amyloidosis look like in an H&E stain?
Definition
smooth, glassy, eosinophilic, homogeneous, extracellular material
Term
What does amyloidosis look like in a congo red stain in light microscopy?
Definition
red-orange deposits
Term
What does amyloidosis look like in a congo red stain in polarization microscopy?
Definition
apple green-yellow birefringence
Term
What is the structure of amyloidosis as seen my transmission electron microscopy?
Definition
straight, criss-crossing fibrils
Term
What is the conformation of amyloidosis as seen in x-ray crystallography?
Definition
cross-β-pleated sheet
Term
What causes amyloidosis?
Definition
overproduction of normal or synthesis of abnormal protein => protein misfolding

if there's insufficent degradation => aggregation into insoluble fibrils & accumulation in tissue
Term
*What is amyloid composed of?
Definition
primarily fibrillary proteins
sm. contribution of glycoproteins
serum amyloid P component
Term
*How many biochemical types are there of amyloid?
Definition
3 major & several minor
Term
What are the 3 major biochemical types of amyloid?
Definition
1) AL (amyloid light chain)
2) AA (amyloid-associated)
3) Aβ (β-amyloid)
Term
What is AL composed of?
Definition
complete or partial Ig light chains, usually λ
Term
What is AA derived from?
Definition
serum amyloid-associated (SAA) protein
Term
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SAA
Definition
an acute phase reactant synthesized by hepatocytes
Term
What is Aβ derived from?
Definition
cleaved fragment of amyloid precursor protein (APP)
Term
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APP
Definition
a neuronal cell surface molecule
Term
What are the 4 minor biochemical types of amyloid?
Definition
1) ATTR (transthyretin amyloid) 2) Aβ2M (β2-microglobulin amyloid) 3) AE (endocrine amyloid) 4) PrP amyloid (prion protein amyloid)
Term
What is ATTR comprised of?
Definition
normal-sequence or variant-sequence transthyretin
Term
def

transthyretin
Definition
serum protein tht binds & transports thyroxine & retinol
Term
What is Aβ2M composed of?
Definition
β2-microglobulin
Term
def β2-microglobulin
Definition
protein in the serum & on the surface of most nucleated cells as a component of MHC I
Term
What is AE derived from?
Definition
protein hormones s.a. amylin, atrial natriuretic factor, calcitonin & insulin
Term
What are the 4 types of systemic amyloidosis?
Definition
1) *Primary
2) Secondary/Reactive
3) Hemodialysis-associated
4) Senile systemic
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
Term
def

dyscrasia
Definition
an abnormal bodily condition
Term
What biochemical type of amyloid is associated with primary amyloidosis?
Definition
AL
Term
What causes secondary/reactive amyloidosis?
Definition
chronic inflammation => long-standing hepatic production of serum amyloid-associated protein (SAA) => protein misfolding

if there's insufficient degradation => amyloid accumulation
Term
What amyloid biochemical type is associated with secondary/reactive amyloidosis?
Definition
AA
Term
What causes hemodialysis-associated amyloidosis?
Definition
long term hemodialysis => retention β2-microglobulin in serum => protein misfolding if there's insufficient degradation => amyloid accumulation
Term
What biochemical type of amyloid is associated with hemodialysis-associated amyloidosis?
Definition
2M
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
Term
What biochemical type of amyloid is associated with senile systemic amyloidosis?
Definition
ATTR
Term
What organ is primarily affected by senile systemic amyloidosis?
Definition
heart
Term
What are the 3 types of localized amyloidosis?
Definition
1) Cerebral
2) Endocrine
3) Other
Term
When is cerebral amyloidosis seen?
Definition
with Alzheimer's & cerebral amyloid angiopathy
Term
What biochemical type of amyloid is associated with cerebral amyloidosis?
Definition
Term
What are 3 forms of endocrine anyloidosis?
Definition
1) Medullary Thyroid Carcinoma
2) Type 2 DM
3) Isolated atrial amyloidosis
Term
What biochemical type of amyloid is associated with endocrine amyloidosis?
Definition
AE
Term
def

Medullary thyroid carcinoma
Definition
malignancy of C cells (parathyroid cells)
Term
Where are associated amyloid deposits derived from in medullary thyroid carcinoma?
Definition
calcitonin
Term
Where is there amyloid accumulation in Type 2 DM?
Definition
in the pancreatic iselts of Langerhans
Term
Where are amyloid deposits derived from in isolated atrial amyloidosis?
Definition
atrial natriuretic factor
Term
What can cause Other localized amyloidosis?
Definition
nodular deposition of amyloid in a wide variety of organs
Term
What are the 2 herediary/familial amyloidosis?
Definition
1) Familiar Mediterranean Fever
2) Familial Amyloidotic Neuropathies
Term
How common are hereditary/familial amyloidosis conditions?
Definition
very uncommon
Term
What causes familial mediterranean fever?
Definition
autosomal recessive disorder of pyrin => systemic accumulation of amyloid
Term
What biochemical type of amyloid is associated with familial mediterranean fever?
Definition
AA
Term
Sx

familial mediterranean fever
Definition
recurrent fever
abdominal & joint pain
serositis (inflammation of serous tissue)
overproduction of IL-1 (chronic inflammation)
Term
What causes familial amyloidotic neuropathies?
Definition
autosomal dominant disorder of transthyretin => deposition of amyloid (esp. in peripheral & autonomic nerves)
Term
What biochemical type of amyloid is associated with familial amyloidotic neuropathies?
Definition
ATTR
Term
What do is the gross characteristic s of organs with amyloidosis in general?
Definition
enlarged, firm & pale
Term
Sx

Amyloidosis
Definition
initially nonspecific => depends on site & extent of organ involvement
Term
*Where does amyloid accumulate in kidney?
Definition
glomeruli, ateries, & interstitium
Term
*What happens due to amyloidosis in the kidney?
Definition
proteinuria & eventual renal failure
Term
Where does amyloid accumulate in the lover?
Definition
initially within the space of Disse => widespread involvement
Term
What leads to widespread amyloid accumulation in the liver?
Definition
pressure atrophy of hepatocytes
Term
What happens to liver function in amyloidosis?
Definition
not usually affected
Term
Where does amyloid accumulate in the spleen?
Definition
Either in the white pulp or red pulp
Term
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sago spleen
Definition
white pulp accumulation of amyloid
Term
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lardaceaous spleen
Definition
red pulp accumulation of amyloid
Term
What happens due to amyloidosis in the spleen?
Definition
increased platelets
Term
What do subendocardial amyloid deposits cause in the heart?
Definition
conduction disturbances & arrythmias
Term
What do myocardial amyloid deposits cause in the heart?
Definition
pressure atrophy of cardiac myocytes & restrictive cardiomyopathy => congestive heart failure
Term
What does amyloid deposition in endocrine organs cause?
Definition
dysfunction
Term
What does amyloid accumulation in the tongue cause?
Definition
macroglossia => speech & swallowing impairment
Term
def

macroglossia
Definition
hypertrophy of the tongue
Term
Where in the skin is especially prone to amyloid deposits?
Definition
eyelids
Term
What happens due to amyloid deposits in the GI tract?
Definition
difficulties with digestion or absorption
Term
Where in the RT can amyloid accumulation occur?
Definition
both upper & lower
Term
Which amyloid biochemical type is most frequently associated with joing & tendon amyloidosis?
Definition
2M
Term
What does amyloidosis in joints & tendons cause?
Definition
carpal tunnel & arthropathy (arthritis)
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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