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19 Cards in this Set
- Front
- Back
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What is amyloid? |
Amyloid is a misfolded protein that deposits in the extracellular space, thereby damaging tissues.
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What are some shared features among all the amyloid proteins?
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(1) beta-pleated sheet configuration
(2) congo-red staining and apple-green birefringence when viewed microscopically under polarized light |
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Primary amyloidosis is ___________ (systemic/local) deposition of AL amyloid, which is derived from ____________.
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systemic; immunoglobulin light chain
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Primary amyloidosis are associated with what?
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Plasma cell dyscrasias like multiple myeloma.
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Secondary amyloidosis is [...]
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Secondary amyloidosis is systemic deposition of AA amyloid, which is derived from serum amyloid-associated protein (SAA).
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Serum Amyloid-Associated protein is an ________________ that is increased in what three states?
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acute-phase reactant; chronic inflammatory states, malignancy and familial mediterranean fever
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Familial mediterranean fever is due to? In what populations do it occur in?
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FMF is due to a dysfunction of neutrophils (autosomal recessive) and occurs in persons of Mediterranean origin.
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FMF presents as what?
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Presents with episodes of fever and acute serosal inflammation (can mimic appendicitis, arthritis, or myocardial infarction). High SAA during attacks deposit as AA amyloid in tissues.
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Clinical findings of systemic amyloidosis include [...]
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(1) Nephrotic syndrome; kidney is the MC organ involved
(2) Restrictive cardiomyopathy/arrhythmia (3) Tongue enlargement (4) Malabsorption (5) Hepatosplenomegaly |
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Diagnosis of systemic amyloidosis?
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Diagnosis requires tissue biopsy. Abdominal fat pad and rectum are easily accessible biopsy targets.
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What can be done with amyloid-damaged organs?
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Damaged organs must be transplanted. Amyloid cannot be removed.
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What is localized amyloidosis?
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Amyloid deposition usually localized to a single organ.
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Localized amyloidosis can occur with age. Provide an example.
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Senile cardiac amyloidosis.
(1) Non-mutated scrum transthyretin deposits in the heart. (2) Usually asymptomatic; present in 25% of individuals > 80 years of age |
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Give an example of a hereditary amyloidosis.
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Familial amyloid cardiomyopathy.
(1) Mutated serum transthyretin deposits in the heart leading to restrictive cardiomyopathy. (2) 5% of African Americans carry the mutated gene |
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Amyloid deposition is seen in Diabetes type I or Diabetes type II?
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Type II. Amylin derived from insulin deposits in islets. Remember that in many cases, the pancreas produces high amounts of insulin.
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In what neurodegenerative disease can we see amyloid deposits?
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Alzheimer's.
Aβ-amyloid (derived from β-amyloid precursor protein) deposits in the brain forming amyloid plaques. |
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This patient will most likely have Alzheimer's by the age of 40. Why?
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β-APP is present on chromosome 21. They have three copies, meaning more β-APP.
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A man on dialysis for several years due to his failing kidneys, develops numbness and "tingling" in the area of his thumb, 1-3 fingers and radial side of his fourth finger. What is going on?
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Entrapment syndrome of the median nerve in the transverse carpal ligament of the wrist due to dialysis-associated amyloidosis. β2-microglobulin deposits in joints, it can't cross the dialysis-filter.
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A man presents with a mass in his neck. Pentagastrin infusion increases calcitonin levels. What could be expected on histology?
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Nests of polygonal cells in an amyloid background. This is medullary CA of the thyroid.
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