Chapter 10 - Amyloidosis Flashcards
Amyloidosis is caused by…
Abnormal aggregation of proteins into β-pleated linear sheets
Systemic types of Amyloidosis
Primary
Secondary
Dialysis-related
Primary Amyloidosis: associated Fibril protein
AL fibrils, derived from immunoglobulin Light chains
Secondary Amyloidosis: associated Fibril protein
Serum Amyloid A (AA)
Dialysis-related Amyloidosis: associated Fibril protein
B2-microglobulin
Primary Amyloidosis is seen in…
Plasma cell disorders like Multiple Mieloma
Secondary Amyloidosis is seen in…
Chronic inflammatory conditions: rheumatoid arthritis, IBD, familial Mediterranean fever, protracted infection
Systemic manifestations of Amyloidosis:
Heart failure
Macroglossia, hepatomegaly
Nephrotic syndrome
Easy bruising, splenomegaly
Neuropathy
Carpal tunnel syndrome
Amyloidosis in Alzheimer’s disease: associated fibril protein
β-amyloid
β-amyloid protein: origin
Amyloid precursor protein (APP)
Amyloidosis in DM2: associated fibril protein
Islet amyloid polypeptide (IAPP)
Amyloidosis causes DM2 by…
Deposition of amylin in pancreatic islets
Amyloidosis in Medullary thyroid cancer: associated fibril protein
Calcitonin
Amyloidosis in Isolated atrial amyloidosis: associated fibril protein
Atrial natriuretic peptide
Age-related amyloidosis: associated fibril protein
Normal (wild-type) transthyretin
Normal (wild-type) transthyretin is seen predominantly in…
Cardiac ventricles
Forms of Hereditary Amyloidosis:
Familial amyloid cardiomyopathy
Familial amyloid polyneuropathies
Hereditary Amyloidosis: associated fibril protein
Mutated transthyretin
Transthyretin gene mutation causes…
Familial amyloid polyneuropathies
Familial amyloid cardiomyopathy is caused by…
Ventricular endomyocardium deposition
What stains and microscopic techniques can be used to visualize amyloid deposits?
Congo red staining, polarized light showing apple-green birefringence, and H&E staining
What findings can be noted on light microscopy in a patient with amyloidosis?
Enlarged tubular basement membranes
Isolated atrial amyloidosis increases the risk of…
Atrial fibrilation