Amyloidosis Flashcards Preview

Pathoma > Amyloidosis > Flashcards

Flashcards in Amyloidosis Deck (22):
1

Amyloid

A misfolded protein that deposits in the extracellular space, thereby damaging tissues

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Characteristics of an Amyloid

Beta pleated sheet configuration
Congo red staining and apple green birefringence when viewed under polarized light

3

Primary Amyloidosis

Systemic deposition of AL amyloid which is dervied from immunoglobulin light chain (kappa and lambda chains). Associated with Plasma cell dyscrasias (overproduction of light chain ex. multiple myeloma)

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Plasma Cell Dyscresias

Overproduction of immunoglobulin light chain that can lead to primary systemic amyloidosis (AL amyloid)

5

AL Amyloid

derived from immunoglobulin light chain

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Apo Serum Amyloid A (SAA)

An acute phase reactant that is increased in chronic inflmmatory states (lupus, rheum arthritis, chrons disease, ulcerative colitis), malignancy and Familial Mediteranean Fever. It deposits as AA Amyloid in tissues

7

AA Amyloid

Derived from SAA which is an acute phase reactant that is increased in chronic inflammatory states, malignancy, and Familial Mediteranean Fever. Systemic amyloidosis 2nd.
Deposits most commonly in kidney.
Dignose via tissue biopsy (ab fat or rectum)
Treat only via transplant

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Clinical Findings of Systemic Amyloidosis

Nephrotic syndrome, restrictive cardiomyopathy, tongue enlargmenet, malabsorption, hepatospleenomegally.

9

FMF

Familail Mediteranean Fever = autosomal recessive dysfunction of neutrophils and occurs in Mediteranean origins. Episodes of fever and acute serosal inflmation (mimics appendicities, arthritis, MI). High SAA during attacks leads to AA amyloid in tissues

10

Localized Amyloid

Amyloid deposition in single organ. Increase in size of organ due to dpeosition of protein can be coutneracted by the fact that amyloid blocks blood vessels and causes atrophy of organ cells.

11

Senile Cardiac Amyloidosis

Non mutated serum transthyretin deposits in the heart. Usually asymptomatic and found in 25% of those odler than 80

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Familial Amyloid Cardiomyopathy

Mutated Serum transthyretin deposits in heart leading to restrictive cardiomyopathy.
5% of African Americans carry the mutated gene

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Non mutated Serum Transthyretin vs Mutated

Non mutated = senile cardiac amyloidosis = asymptomatic = old
Mutated = Familial Amyloid Cardiomyopathy = restrictive heart walls= african americans

14

Non Insulin-dependent Diabetes Mellitus (Type II)

Amylin derived from insulin deposits in the islets of the pancreas

15

Alzheimer Disease

A"beta" amyloid depositis in the brain forming amyloid plaques. Gene for Beta-APP is present on chromosome 21 so see early alzheimers in most down syndrome/trisomy 21

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Down Sydrome

Trisomy 21, chromosome with Beta APP gene so more likely to get ABeta amyloid = alzheimers

17

Dialysis Assoicated Amyloidosis

Beta2 microglobulin is a structural component of MHC1 and is not easily filtered out in dialysis and so often deposits in joints

18

Beta2 microglobulin

Structural compenet of MHC1 and deposits in joints in dialysis patients. Dialysis Assoicated Amyloidosis

19

Medullary Carcinomaof the thyroid

Calcitonin (produced by tumor cells) deposits within the tumor. Get "tumor cells in amyloid background)

20

"Tumor cells in amyloid background"

Medullary carcinoma of Thyroid as a result overproduction and deposition of calcitonin.

21

Familial Amyloidotic Polyneuropathy

Autosomal dominant disorder in transthyretin gene. Misfodled protein deposits in peripheral and autonomic nerves. More common in Sweden, Portugal, and Japan

22

APrP Amyloid

Lead to spongiform encephalopathies. Amyloid is dervied from prion proteins. Can get Kuru, Creutzfeldt-Jakob disease, Gerstmann-Straussler-Sheinker disease, scrapie, and bovine spongiform encephalopathy