Nelson- Amyloidosis Flashcards Preview

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Flashcards in Nelson- Amyloidosis Deck (40)
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1

Define amyloidosis

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

2

What is the characteristic appearance of amyloid in tissue sections?

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



3

What special stain is used to view amyloidosis?

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

Congo red- appears red

On polarization= red stained amyloid exhibits green birefringence

4

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

Group of disorders that have amyloid deposited in places

5

How does amyloid injure adjacent cells?

Deposited amyloid--> tissue and organ dysfunciton

6

What are the 5 types of amyloid protein?

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

7

AL- pathological mechanism

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

8

AL- precursor proteins

complete Ig light chains (lamda L)
amino terminal fragments of L chains or both

9

AA- pathological mecahnism

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

**SAA production increased in inflammatory conditions> chronic inflammation

10

AA- precursor proteins

Proetolysis of serum amyloid protein (SAA)

often associated w/ high density lipoprotein

11

AB- precursor proteins

Proetolysis of amyloid precursor protein

12

AB- pathological mechanism

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

13

TTR- precursor protein

Mutated TTR

Normal serum protein that binds and transports thyroxine and retinol

14

TTR- pathological mechanism

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

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

15

B2 microglobulin- precursor protein

normal serum protein

16

B2 microglobluin- pathological mechanism

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

(hemodialysis associated amyloidosis)

17

What can cause localized amyloidosis?

prion diseases>
accumulation in CNS

18

What is the mechanism for amyloidosis?

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

(classified by type of protein produced)

19

Define systemic amyloidosis

involving several organ systems

20

Define localized amyloidosis

involving a single organ (heart)

21

Define primary amyloidosis

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

22

Define secondary amyloidosis

complication of an underlying chronic inflammatory process

23

Define hereditary/familal amyloidosis

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

24

Describe primary amyloidosis.

AL- most common type in the US

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

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

Monoclonal free L chain>
deposition

26

Describe reactive systemic amyloidosis.

2nd most common type

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

27

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

AA is most common b/c of chronic infectious disease

28

AA (reactive) is associated w/ what cancers?

REneal cell carcinoma

Hodgkin lymphoma

29

Describe hemodialysis associated amyloidosis.

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

30

Describe Heredofamilial amyloidosis (two types)

Mutated TTR (heritable/cardiomyopathic amyloidosis)>
amyloid deposition

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