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33 Cards in this Set
- Front
- Back
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What is amyloidosis?
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this is extracellular deposits of misfolded proteins
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What is the special stain for amyloidosis? what does it stain?
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Congo red stain
this stain B pleated sheets |
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What is used to radiographically determine the extent of amyloid deposits?
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Serum amyloid P component, a stabilizing protein
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What does amyloidosis do to affected tissues?
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this causes misfolded protein deposition- causes affected tissues to become hardened and waxy
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What are the clinical signs of amyloidosis?
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diffuse organomegaly, with evidence of organ dysfunction
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How much does Serum amyloid P component make up of a amyloidosis deposit?
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Less than 5%
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What makes up AL amyloidosis?
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this is formed from monocolonal Ig light chain, K-type, or lambda-type
From monoclonal plasma cell proliferations |
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What causes AL amyloidosis?
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Primary amyloidosis
Amyloidosis asscoaited with multiple myeloma (plasma cell tumors) |
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What organs are most affected in AL amyloidosis?
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widely deposited in connective tissue.
Heart Liver Spleen Kidneys |
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What is a clinical feature of AL amyloidosis involving the eyes?
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raccoon eyes, due to local coagulation factor X deficiency
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What protein causes secondary/reactive systemic amyloidosis?
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SAA (serum amyloid associated protein)
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What produces SAA? and why?
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this is acute phase reactants produced by the liver.
due to chronic inflammatory conditions |
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What are the main causes of reactive systemic amyloidosis
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infections like TB, osteomyelitis, Bronchiectasis
Noninfectious inflammatory causes like: rheumatoid arthritis or chronic skin popping of narcotics |
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What organs are most affected in reactive systemic amyloidosis?
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Liver
Spleen kidneys |
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What disease is A-Beta Amyloid associated with?
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Alzheimers disease, cerebral amyloid angiopathy
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What is A-beta amyloid composed of?
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Amyloid Precursor Protein (APP) complexed with Apo E
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What organ is most involved with ATTR amyloid?
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the heart- causes senile cardiac amyloidosis
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What makes up ATTR?
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this is derived from transthyretin (TTR) and Prealbumin
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What is the molecule implicated in A-beta-2m amyloidosis?
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Beta-2 microgloublin, which is an MHC class 1 molecule
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Who usually gets A-beta-2m amyloidosis?
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this accumulates in pts on long term dialysis for endstage renal disease
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Where does A-beta-2m amyloidosis usually present?
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in joints, synovium, tendon sheaths- gives carpal tunnel syndrome
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What type of amyloidosis causes polyneuropathies?
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this is due to accumulation of mutant ATTR
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What is Familial Mediterranean Fever?
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this is an autosomal recessive disease.
causes the accumulation of AA (an acute phase reactant produced by the liver-derived from serum amyloid associated protein) |
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What are common locations of localized amyloidosis, and what type of amyloidosis is it?
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this is AL type
usually creates deposits in the: Lung, larynx, skin, bladder, tongue, and periorbital regions |
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What kind of amyloid occurs in type II DM, and where?
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this produces AIAPP (islet amyloid peptide)
this cause islet cell tumors |
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What kind of amyloid occur in the thyroid, and where in the thyroid?
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medullary carcinoma of the thyroid
from C-cells, called Acal |
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What type of amyloid is the senile amyloid?
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ATTR
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What are the two systemic forms of Amyloid?
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AL (from monoclonal immunoglobulin light chains)
AA (from acute phase reactants produce by the liver called SAA) |
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What happens to the spleen during amyloidosis?
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this enlarges quite a bit. up to 800gm, when the normal is 150gm.
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what does amyloidosis do to the liver?
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hepatomegaly
this affects the sinusoids too- causes pressure atrophy of hepatocytes |
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What two types of amyloidosis typically affect the heart the most?
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primary AL (light chains of immunoglobulins)
and senile cardiac amyloidosis (ATTR) |
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Where do you do an aspiration and stain to look for systemic amyloidosis?
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abdominal fat- very specific, low sensitivity
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What type of amyloidosis has the worst prognosis?
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myeloma associated
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