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Pathology Unit 2: Amyloidosis, Skin, Vasculitis, Joints, Autoimmune > Amyloidoses > Flashcards

Amyloidoses Flashcards

1
Q

what is the stain commonly used to diagnose amyloidosis?

A

Congo red stain

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2
Q

what is the tertiary structure of amyloid protein?

A

beta pleated sheet

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3
Q

what type of amyloid is usually associated with monoclonal B-cell dyscrasias?

A 
AL amyloid
(these used to be called primary amyloidosis)
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4
Q

what amyloid producing disease is characterized by PMN leukocyte disfunction and recurrent episodes of serositis?

A

Familial Mediterranean Fever

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5
Q

what type of amyloid is featured in familial Mediterranean fever and renal cell carcinoma?

A

AA amyloid

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6
Q

what type of amyloid is seen in Alzheimer’s?

A

A beta amyloid

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7
Q

what protein is the precursor for amyloid deposit in Islets of Langerhans in type 2 diabetics?

A

amylin (AKA islet amyloid polypeptide) becomes AIAPP

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8
Q

what is the precursor protein for amyloid deposited in joints of chronic dialysis patients?

A

beta 2 microglobulin (invariant chain of MCH class I)

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9
Q

what is the precursor protein for amyloid assoc with Creutzfeldt-Jacob disease?

A

prion protein (PrPsc)

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10
Q

what is the amyloid precursor protein for familial amyloidotic polyneuropathy?

A

transthyretin (TTR)

- most common hereditary amyloidosis

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11
Q

what is a definitive form of treatment for familial amyloidotic polyneuropathy?

A

liver transplant

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12
Q

what cell type mediates amyloid turnover?

A

macrophages

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13
Q

what type of heart disease is generally associated with amyloidosos?

A

restrictive cardiomyopathy: diastolic dysfunction and preserved systolic function

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14
Q

what type of amyloid is assoc with Waldenstrom macroglobulinemia?

A

AH amyloid

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15
Q

what type of protein is found in the urine of pts with plasma cell dyscrasia and amyloidosis?

A

Bence-Jones proteins

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16
Q

How is clinical amyloidosis classified?

A

by the identity of the fibril protein accumulating

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17
Q

amyloid-forming proteins can exist in what stable structures?

A

1 - native form

2 - beta sheets

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18
Q

what form of amyloid-forming proteins can aggregate to form fibrils?

A

beta sheets

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19
Q

what biophysical properties do all amyloid fibrils share?

A

1 - insoluble in physiologic solutions
2 - fairly resistant to proteolysis
3 - binds congo red dye to create green birefringence

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20
Q

What is the risk of an abnormally high abundance of certain proteins that are present in low concentrations normally?

A

AMYLOID Deposition:
ex. serum amyloid A protein (chronic inflammation)
beta2 microglobulin (renal failure)

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21
Q

True or False: Normal concentrations of proteins with amyloidogenic potential will not produce amyloidosis

A

False, if proteins with amyloidogenic potential are present for a long period of time at normal concentrations they can aggregate as seen in senile amyloidosis (transthyretin) and Alzheimer’s (beta protein)

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22
Q

Can amyloidoses be acquired or inherited?

A

Yes, ex. monoclonal immunoglobulin light chains in AL amyloidosis and genetic amyloidogenic variants of transthyretin, lysozyme, apolipoprotein AI, and fibrinogen A alpha chain

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23
Q

True or False: amyloid deposits are made solely of amyloid

A

False: they contain minor constituents such as GAGs, serum amyloid protein, apoE, laminin, collagen IV

24
Q

True or False: Females over 40 are most likely to have wild type transthyretin accumulate

A

FALSE: MEN OVER 60 make of 90% of the patient population for this disorder

25
Q

True or False: the pathologic effects of amyloid are due to inflammatory responses?

A

FALSE! amyloid provokes little to no inflammatory reaction

they impair function by physically obstructing things (not just large deposits)

26
Q

Does the amount of amyloid deposited indicate severity of dysfunction?

A

NO, it varies depending on person, organ and rate of deposition.

27
Q

How can we treat amyloidoses?

A

reduce the supply of amyloid precursors!

28
Q

What methods are used to detect amyloid?

A

1 - congo red stain

2 - immunohistochemistry (depends on fibril protein type)

29
Q

all forms of amyloid consist of _________ ________ of ____________ arrays of fibrils.

A

all forms of amyloid consist of INTERLACING BUNDLES of PARALLEL arrays of fibrils

30
Q

What is the most common acquired amyloidosis?

A

systemic AL amyloidosis

31
Q

What type of amyloidosis may result from chronic infections or inflammatory diseases?

A

AA amyloidosis

why? long-term overproduction of acute phase reactant SAA

32
Q

what part of SAA forms the amyloid fibrils?

A

N-terminal cleavage fragment = single non-glycosylated polypeptide chain

33
Q

What infections may cause AA amyloidosis?

A

TB, leprosy

34
Q

What types of cell convert SAA into AA?

A

macrophages cleave SAA into AA via several proteinases

35
Q

What is the key to treating AA amyloidosis?

A

suppress the underlying inflammatory disorder

36
Q

Why is a rectal biopsy used to diagnose AA amyloidosis?

A

the AA amyloid deposits are widely distributed

37
Q

What are the transcriptional regulators of SAA?

A

IL-1, IL-6, TNF-alpha

38
Q

Which of the following organs may be directly affected by AL amyloidosis?
Brain, Heart, Liver, Kidneys, Peripheral nerves

A

Virtually all organs EXCEPT the brain can be directly affected but those listed are most important clinically (except the brain)

39
Q

Which amyloidosis involves immunoglobulins?

A

AL amyloidosis: consists of the variable region of Ig light chains derived from kappa or lambda polypeptides

40
Q

ATTR amyloidosis almost always presents with ________ _________.

A 
Restrictive cardiomyopathy 
(ATTR = transthyretin amyloidosis)
41
Q

Medullary carcinoma of the thyroid is associated with amyloid precursor?

A

procalcitonin (becomes ACal)

42
Q

Where in the brain are amyloid deposits never seen?

A

cerebral parenchyma (not seen in any form of acquired systemic visceral amyloidosis)

43
Q

What protein is beta-protein derived from and where is the precursor found?

A

amyloid precursor protein (APP), transmembrane protein in neurons and glia

44
Q

What is the link between Alzheimer’s and Down Syndrome?

A

APP (which makes beta amyloid, which causes Alzhiemer’s) is encoded on chromosome 21; some Down syndrome patients develop AD by 35

45
Q

What hereditary amyloidosis presents as cerebral amyloid angiopathy with recurrent cerebral hemorrhage and silent systemic deposits?

A

cystatin C amyloidosis

- only reported in Icelandic families

46
Q

most common hereditary renal amyloidosis

A

Hereditary fibrinogen A alpha-chain amyloid

47
Q

A 50 year old man presents with proteinuria, and hypertension. Despite your best efforts to treat him, he develops end-stage renal failure within 5 years. What is the most likely amyloidosis in this patient? And what is the mechanism of disease?

A

Hereditary fibrinogen A alpha-chain amyloid.

Mech: amyloid deposits in kidneys, spleen, and perhaps liver

48
Q

Because amyloid deposits accumulate along _______ __________, the deposits take on the architectural framework of the ________ ________.

A

because amyloid deposits accumulate along STROMAL NETWORKS, the deposits take on the architectural framework of the ORGANS INVOLVED

49
Q 
Amyloid deposition can cause: 
A- Increased size of organs affected
B - Organ Atrophy
C - All of the above
D- none of the above
A

C: amyloid add material to th organs where they deposit but they may also occulde blood vessels and impair circulation leading to atrophy

50
Q

Where do amyloid deposits tend to localize? and how does this affect cellular processes?

A

they localize between parenchymal cells and their blood supply interfering with normal nutrition and gas exchange

51
Q

What are two methods through which amyloidosis damages/ kills cells?

A

1 - blocking blood supply

2 - direct toxic effect through protofibrils interacting with cell membranes

52
Q

What classic symptoms of amyloidosis should instantly indicate the diagnosis?

A

TRICK QUESTION! There is no single set of sx that point to amyloidosis
- sx depend on underlying disease, type of amyloid and organ affects

53
Q

Enlargement of the tongue is associated with what type of amyloidosis?

A

AL amyloidosis

54
Q

What type of amyloid is associated with spongiform encephalopathies?

A

PrPsc (prion protein)

55
Q

what type of amyloid protein is associated with multiple myeloma?

A

AL amyloid (immunoglobulin precursor)

Pathology Unit 2: Amyloidosis, Skin, Vasculitis, Joints, Autoimmune (5 decks)

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