Chapter 1 Growth Adaptations, Cellular Injury, and Cell Death Flashcards
Amyloid
Misfolded protein deposits in extracellular space
Biopsy is diagnostic
Structure
Beta pleated sheet,
Stains Congo red and shows background Green birefringence (refraction depending on polarization of light)
Primary Systemic Amyloidosis
- AL amyloid from light chain of immunoglibulin. Chronic inflamation leads to AL amyloid formation
- Plasma Cell dyscrasis (malignant proliferation of plasma cells (multiple myeloma))
Secondary Systemic Amyloidosis
- Derived from SAA (Serum Amyloid Associated)
- Increased in chronic inflammation, malignancies, and familial hereditery fever (neutrophil defect), on serosal surfaces (can mimic appendicitis and myocardial infarction)
- Nephrotic syndrome, restrictive cardiomyopathy, tongue enlargment, malabsorption, hepatosplenomegaly.
Localized Amyloidosis
- Senile cardiac amyloidosis (nonmutated serum transthyretin, often asymptomatic, common)
- Familial Amyloid Cardiomyopathy (mutated Transthyretin, 5% African Americans)
- Type 2 Diabetes - Amylin deposits in pancreas (insulin byproduct)
- Alzhimers - AB amloid from APP, crhomosome 21 (downsyndrome)
- Dialysis Associaed Amyoidosis - B2 microglobulin not filtered by dialysis (deposits in joints)
- Medullary Carcinoma of Thyroid - C-cells, excess calcitonin production results in amyloid (diagnosis is tumor cells in a background of amyloid)
Senile Cardiac Amyloidosis
-Non mutated Transthyretin deposits in heart. Assymptomatic and common in elderly
Familial amyloid cardiomyopathy
-mutated transthyretin deposits in Heart. Common in African Americans. Results in restrictive Cardiomyopathy
Type 2 diabetes
-Overproduction of insulin results in amylin deposits in pancreatic islets (byproduct of insulin production)
Dialysis Amyloidosis
-B2 microglobulin not filtered out in dialysis, deposits in joints
Alzheimers
AB amyloid from APP, located on Chromosome 21 (downs have alzheimers)
Medullary Carcinoma of Thyroid
-C-cell neoplasm secretes excess calcitonin resulting in amylid deposition. Appears as tumor cells on amyloid background.