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17 Cards in this Set
- Front
- Back
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Amyloid |
Proteinaceous substance (fibrillar) that forms abnormally in extra cell space of organs
Result= tissue atrophy and damage |
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Pathogenesis? |
Abnormal protein folding |
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Physical properties of amyloidosis |
Non-branching fibrils Cross beta-pleated sheet conformation |
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Chemical properties |
95% fibril proteins, 5% glycoprotein (P Component) P Component bound to all fibrils |
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2 Classifications |
Systemic- more than one organ system Localized- limited to single organ |
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Primary Amyloidosis or AL Amyloid |
Misfolded protein= Ig Light Chain Produced by B cells Systemic Example- produced in multiple myeloma |
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Reactive Systemic Amyloidosis |
Misfolded protein- AA protein derived from Serum Amyloid Associated protein that is made in liver (SAA) SECONDARY- most have other underlying issue(RA, TB, Inflammm bowel) |
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AMyloid of Aging (Senile systemic Amyloidosis- SSA) |
Protein misfolded- Transthyretin (TTR) Mutated TTR responsible for other hereditary amyloidosis, but normal in SSA Age-dependent, asymptomatic, incidentally found (Heart, GI, lungs) |
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Hemodyialysis Associated Amyloidosis |
Misfolded protein- Beta2-microglobin Patients on long term dialysis- not filtered |
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Symptoms |
Variable- general symptoms= fatigue, weight loss, syncope Cardiac- arrhythmia, SOB Renal- proteinuria, edema GI- malabsorption, diarrhea, dysphagia |
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3 Possible reasons/ways proteins deposit |
1. Increased concentration= then have tendency to fold improperly 2. mutant proteins= structurally unstable 3. Proteolytic cleavage and fragmentatoin |
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General tissue damage |
Protein buildup causes- pressure atrophy, loss of abnormal tissue and sometimes loss of function |
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Kidney |
small, lrg, norm deposits usually in glomeruli |
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Heart |
Enlarged, deposits in subendocardial and myocardial |
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Liver |
Normal or enlarged deposits in Space of Disse and vasc |
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Prognosis |
Systemic= poor AL disease w/ multi myeloma= med survival 2 yrs Reactive type= slightly better survival |
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Treatment |
Treat early to prevent organ damage treat underlying disorder clonal disorders of plasma= chemo inflammatory disorders (reactive type)- anti-inflam
New treatment= target proteins/folding
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