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13 Cards in this Set
- Front
- Back
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What are the properties associated with ß pleated sheets of amyloid? |
Formation of non-branching fibrils that deposit between the cells of various organs Insoluble Resistance to proteolysis Stain with Congo Red and are Apple Green under polarized light |
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What is the chemical nature of amyloid? |
Major component is protein fibrils Minor comonpent is "p" component - normal serum glycoprotein Glycosaminoglycans |
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What are the three major proteins that from amyloid? |
AL - Immunoglobulin light chains (variable regions of lambda and kappa fibrils) AA - acute phase protein produced during inflammatory conditions AF - Prealbumin |
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How does AL amyloid form? |
Incomplete digestion of IgG is misfolded with circulating p component and binds to proteoglycan that deposits in any tissue. |
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How can amyloid be diagnosed? |
By biopsy and staining is the only way |
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What is the etiology of AL amyloidosis? |
Plasma cell malignancy that leads to production of fragment Ig light chains which are conjugated with P component and deposit in various tissues
20% associated with multiple myeloma (plasma cell cancer) |
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What are the clinical features of AL amyloidosis? |
Relatively rare disease Median age of onset is 75-80 Symptoms commonly fatigue and weight loss, but also CHF, nephrotic syndrome, malabsorption, neuropathy PE shows hepatomegaly, purpura, edema, macroglossia |
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What are the major sites of AL amyloid deposits?
What are the major sites of AA amyloid deposits? |
AL - heart, GI, tongue, skin, nerves
AA - liver, spleen, kidneys, adrenal glands |
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Where does amyloid deposit in the kidneys?
What are the early and late lesions of renal amyloidosis? |
Mainly in the glomeruli, but also in tubules and blood vessels
Early: thickening of mesangial matrix, widening of basement membrane Late: obliteration of capillary lumen and glomerulia MAJOR CAUSE OF MORBITIDY |
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What are the patterns of deposit in the spleen? |
Sago spleen - deposits in white pulp
Lardaceous spleen - deposits first in red pulp, then white |
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What are the patterns of deposit in the liver? |
Early: space of Disse, progressive encroachment on hepatic parenchyma Late: complete replacement of hepatic parenchyma, vascular involvement and Kuppfer cell deposition, though liver still functions normally. |
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What happens when amyloid deposits in the heart? |
Restrictive cardiomyopathy (stiff ventricular wall) |
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What is the current treatment recommendation for amyloidosis? |
High dose chemotherapy and stem cell transplant. |
