Path - Inflammation (Amyloidosis & Lipofuscin) Flashcards
Pg. 227-228 in First Aid 2014 Sections include: -Amyloidosis -Lipofuscin
What is amyloidosis? What 2 major effects does it have?
Abnormal aggregation of proteins (or their fragments) into Beta-pleated sheet structures => damage and apoptosis
What are 6 common types of amyloidosis?
(1) AL (primary) (2) AA (secondary) (3) Dialysis-related (4) Heritable (5) Age-related (senile) systemic (6) Organ-specific
What is the amyloid in AL amyloidosis? Is this primary or secondary amyloidosis?
Due to deposition of proteins from Ig Light chains; AL (primary); Think: “aL = ig Light chains”
What is the amyloid in AA amyloidosis? Is this primary or secondary amyloidosis?
Fibrils composed of serum Amyloid A; AA (secondary); Think: “AA = serum Amyloid A”
What are 2 conditions in which AL amyloidosis can occur?
Can occur as a plasma cell disorder or associated with multiple myeloma
To what extent does AL (primary) amyloidosis usually affect the body? Give 5 examples.
Often affects multiple organ systems, including (1) renal (nephrotic syndrome), (2) cardiac (restrictive cardiomyopathy, arrhythmia), (3) hematologic (easy bruising), (4) GI (hepatomegaly), and (5) neurologic (neuropathy)
In what type of conditions is AA (secondary) amyloidosis seen? Give 4 examples of such conditions.
Seen with chronic conditions, such as rheumatoid arthritis, IBD, spondyloarthropathy, protracted infection
How does AA (secondary) amyloidosis compare/contrast to AL (primary) amyloidosis in terms of its effects/extent?
Often multisystem like AL amyloidosis
What is the amyloid in dialysis-related amyloidosis? What are 2 patient populations/settings in which this may occur?
Fibrils composed of Beta2-microglobulin in patients with ESRD and/or on long-term dialysis
How may dialysis-related amyloidosis present?
May present as carpal tunnel syndrome
Briefly describe heritable amyloidosis (in terms of how it is defined).
Heterogenous group of disorders
What is an example of heritable amyloidosis? What causes this?
Example is ATTR neurologic/cardiac amyloidosis due to transthyretin (TTR or prealbumin) gene mutation
What amyloid causes age-related (senile) systemic amyloidosis, and where?
Due to deposition of normal (wildtype) TTR in myocardium and other sites
How does age-related (senile) systemic amyloidosis compare to AL amyloidosis in terms of progression of cardiac dysfunction?
Slower progression of cardiac dysfunction relative to AL amyloidosis
What is the definition of organ-specific amyloidosis?
Amyloid deposition localized to a single organ
What disease is the most important form of organ-specific amyloidosis, and what is the amyloid?
Most important form is amyloidosis in Alzheimer disease due to deposition of amyloid-Beta protein cleaved from amyloid precursor protein (APP).
What kind of amyloidosis is Islet amyloid peptide (IAPP)? In what condition is it commonly seen, and what causes it?
Organ-specific; Islet amyloid polypeptide (IAPP) is commonly seen in diabetes mellitus type 2 and is caused by deposition of amylin in pancreatic islets
What stain shows amyloidosis? What special visualization technique can be used, and how does amyloidosis appear in this case?
Congo red stain shows amyloid deposit; Congo red stain shows apple green birefringence under polarized light
What is lipofuscin? With what process is it associated?
A yellow-brown “wear and tear” pigment associated with normal aging
What mechanism(s) form(s) lipofuscin?
Formed by oxidation and polymerization of autophagocytosed organellar membranes
In autopsy of what patient population is lipofuscin seen, and where in the body?
Autopsy of elderly person will reveal deposits in heart, liver, kidney, eye, and other organs
