8. Amyloidosis Flashcards
What is amyloidosis?
A group of diseases characterized by the deposition of an extracellular protein that has specific properties.
Describe an amyloid.
Extracellular protein that tends to form beta-pleated shets and stain red with apple green birefringement with Congo red stain. It is composed of 3 elements: fibrillary protein, amyloid P component, and glycosaminoglycans (heparin sulfate). The specific composition of the protein varies with each disease producing amyloidosis.
What is primary amyloidosis?
Complicates plasma cell disorder (e.i., multiple myeloma, B-cell lymphomas, etc.), the amyloid protein is AL, and the fibrillary protein is kappa or lambda light chains.
What is another name for secondary amyloidosis and what is it?
AKA Reactive amyloidosis, the type of amyloid is AA and the fibrillary protein is serum amyloid A (SAA). SAA is an acute phase reactant produced by the liver and is elevated with ongoing inflammation and neoplasia seen in diseases such as Rheumatoid arthritis (RA), SLE, TB, osteomyelitis, Crohn’s, cancer, etc.
What is Familial Mediterranean fever?
A reactive amyloidosis with amyloid AA and serum amyloid A (SAA). It is autosomal recessive characterized by recurrent inflammation, fecer, and neutrophil dysfuntion.
What is hemodialysis-associated amyloidosis?
A reactive amyloidosis with amyloid A-beta-2M, with Beta2-microglobulin for fibrillary protein that is seen in patient with chronic treatment with hemodialysis. May cause carpal tunnel syndrome and joint disease.
What is senile cerebral amyloidosis?
AKA Alzheimer disease, it is a localized type of amyloidosis with A-Beta amyloid with Beta-amyloid precursor protein (BetaAPP) as fibrillary protein. It is found in Alzhiemer plauqes in the cerebral vessels.
Where is the gene for BetaAPP is located?
Chromosome 21.
What is senile cardiac amyloidosis?
A localized type of amyloidosis with ATTR amyloid from transthyretin fibrillary protein. It is seen in men above 70 years, and may cause heart failure.
What are three endocrine type localized amyloidosis?
I. Medullary carcinoma of the thyroid (procalcitonin). II. Adult-onset diabetes (amylin). III. Pancreatic islet cell tumors (amylin).
What is the most commonly involved organ in a systemic amyloidosis?
Kidney
What are the effects of systemic amyloidosis in the kidney?
Nephrotic syndrome that leads to progressive renal failure.
What are the effects of systemic amyloidosis in the GI tract?
Tongue enlargement and malabsorption. May also cause hepatospleenomegaly.
What are 4 effects of systemic amyloidosis in the heart?
I. Restrictive cardiomyopathy. II. Low voltage EKG. III. Cardiac arrhythmias. IV. CHF
