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IHO Week 6 > Nelson- Amyloidosis > Flashcards

Nelson- Amyloidosis Flashcards

1
Q

Define amyloidosis

A

group of disorders where amyloid is deposited in extracellular space of tissues and organs> tissue organ and dysfunction

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2
Q

What is the characteristic appearance of amyloid in tissue sections?

A

Pathological proteins w similar physical properties (4-6 fibrils wound around one another)

Linear non branching fibrils in cross beta pleated sheet configuration > characteristic staining

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3
Q

What special stain is used to view amyloidosis?

A

H&E- amorphous, eosinophillic, hylaline, extracelluar substance–> pressure atrophy of adjacent cells

Congo red- appears red

On polarization= red stained amyloid exhibits green birefringence

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4
Q

Is amyloidosis a single disease entity or a group of diseases?

A

Group of disorders that have amyloid deposited in places

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5
Q

How does amyloid injure adjacent cells?

A

Deposited amyloid–> tissue and organ dysfunciton

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6
Q

What are the 5 types of amyloid protein?

A 
AL- amyloid light chain
AA- amyloid associated
AB- beta amyloid protein
TTR- transthyretin
B2- microglobluin
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7
Q

AL- pathological mechanism

A

monoclonal population of plasma cells>
produces free Ig light chain protein>
AL

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8
Q

AL- precursor proteins

A

complete Ig light chains (lamda L)

amino terminal fragments of L chains or both

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9
Q

AA- pathological mecahnism

A

Increased SAA production + enzyme defect>
incomplete SAA break down>
deposition

**SAA production increased in inflammatory conditions> chronic inflammation

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10
Q

AA- precursor proteins

A

Proetolysis of serum amyloid protein (SAA)

often associated w/ high density lipoprotein

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11
Q

AB- precursor proteins

A

Proetolysis of amyloid precursor protein

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12
Q

AB- pathological mechanism

A

Found in cerebral plaques and walls of cerebral vessels in Alzheimers disease

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13
Q

TTR- precursor protein

A

Mutated TTR

Normal serum protein that binds and transports thyroxine and retinol

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14
Q

TTR- pathological mechanism

A 
Mutated TTR (Heritable neuropathic/cardiomyopathic amyloidosis) >
amyloid deposition

Non-mutated TRR (senile systemic amyloidosis) > TRR deposition in heart of aged pts

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15
Q

B2 microglobulin- precursor protein

A

normal serum protein

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16
Q

B2 microglobluin- pathological mechanism

A

Cannot be filtered through dialysis membranes>
accumulates in long term dialysis

(hemodialysis associated amyloidosis)

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17
Q

What can cause localized amyloidosis?

A

prion diseases>

accumulation in CNS

18
Q

What is the mechanism for amyloidosis?

A

abnormal protein folding into fibrils>
increased resistance to normal degradation>
accumulation of mysfolded protein

(classified by type of protein produced)

19
Q

Define systemic amyloidosis

A

involving several organ systems

20
Q

Define localized amyloidosis

A

involving a single organ (heart)

21
Q

Define primary amyloidosis

A

associated w/ an immunocyte (plasma cell, B cell disorder)

22
Q

Define secondary amyloidosis

A

complication of an underlying chronic inflammatory process

23
Q

Define hereditary/familal amyloidosis

A

group of heterogenous heritable forms of amyloidosis w/ several distinctive patterns of organ involvement

24
Q

Describe primary amyloidosis.

AL- most common type in the US

A

Monoclonal proliferation of plasma cells>
production of monoclonal IgL/Ig H chains (w/out H chain= bence jones protein)>
AL/AH>
systemic>
heart, kidney, periohperal nerve, GI tract

25
Q

What type of light chain deposition is seen in pts w/ multiple myeloma?

A

Monoclonal free L chain>

deposition

26
Q

Describe reactive systemic amyloidosis.

2nd most common type

A

Chronic inflammatory condition (RA), ct disorder, inflammatory bowel disorder>
serum AA>
AA amyloidosis (high density apolipoproteins)>
systemic>
kidney, liver, spleen

27
Q

What is the most common type of amyloidosis in the developing world and why?

A

AA is most common b/c of chronic infectious disease

28
Q

AA (reactive) is associated w/ what cancers?

A

REneal cell carcinoma

Hodgkin lymphoma

29
Q

Describe hemodialysis associated amyloidosis.

A 
Prolonged periods of dialysis to treat end stage renal disease>
B2 microglobulin>
Ab2m>
deposition of B2 microglobulin>
bone structures
30
Q

Describe Heredofamilial amyloidosis (two types)

A 
Mutated TTR (heritable/cardiomyopathic amyloidosis)>
amyloid deposition

Non-mutated TTR (senile systemic amyloidosis)>
TTR deposition in heart of aged pts

31
Q

Describe age related systemic amyloidosis

A

Cardiac amyloidosis d/t normal TTR

Normal TTR protein>
deposition in heart>
cardiomyopathy and arrhythmia

*elderly 70-80s

32
Q

What is a hereditary type of amyloidosis commonly seen in african americans?

A

Mut in Ile22 indicates cardiac amyloidosis d/t mut TRR

mut allele commonly seen in African americans-> screen for this

33
Q

Describe localized (organ specific) amyloidosis

A

Nodular deposition in lung, larynx, skin, urininary bladder, tongue, orbi

*limited to 1 organ/tissue

most common: amyloid plaques and amyloid laden cerebral vessels in AD

34
Q

What precursor protein is associated w/ alzheimers and where is it distributed?

A

APP> AB

cerebral plaques
cerebral vessels

35
Q

What organ systems are most commonly affected by systemic amyloidosis?

A

kidneys, heart , liver

36
Q

What are some of the key clinical sxs associated w/ systemic amyloidosis?

A 
waxy skin
easy bruising
enlarged muscles
heart failure
cardiac conduction abnormalities
heptatomegaly
renal dysfunciton
peripheral/autnomic neuropathy
impaired coagulation (factor X def)
37
Q

How do you dx amyloidosis/

A

Gold standard: tissue biopsy w/ amyloid stainging

38
Q

How do you dx suspected systemic disease?

A

fat pad aspiration (from abdomen) or rectal biopsy

39
Q

How do you determine the type of ayloid present?

A

immunohistochemistry
liquid chromatography
mass spec

40
Q

How do you treat amyloidosis?

AA
AL
Dialysis realted
herediatry forms w/ TTR

A

Varies w/ amt of amyloid produced

AA- target underlying infection/inflamm disorder
AL- underlying PC disorder
Dialysis related- alter mode of dialysis or renal transplant
herediatry forms w/ TTR- liver transplant

IHO Week 6 (14 decks)

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