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The Amyloidoses Douglas A. Stahura 4/7/99
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Amyloidoses History Virchow described a homogenous eosinophilic material which binds Congo red stain. Associated with inflammatory joint disease and amyloid deposition in kidney, liver, spleen.
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Amyloidoses Definition
Criteria for definition Homogenous, hyaline eosinophilic on H+E stain Crystal violet metachromasia Stains with alkaline Congo red Apple green birefringence on polarized light Fibrillar structure on microscopy Salt extraction yields p-component forms Fibril is unique for all forms of amyloid
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Amyloidoses AL L-chain primary, MM AH H-chain primary, MM
ATTR transthyretin senile systemic Abeta2M Beta2 microglobulin dialysis Abeta beta-protein precursor Alzheimer Ascr Scrapie Creutzfeldt-Jakob
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Amyloidoses Most common clinical form beta protein placque of Alzheimer’s for rheumatoligist AL disease primary amyloidoses or MM
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Amyloidoses Clinical Presentation
Renal: nephrotic range protenuria or mild must be distinguished from monoclonal light chains protein may diminish with renal insufficiency HTN Enlargement occurs early
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Amyloidoses Clinical Presentation
Cardiac: Restrictive cardiomyopathy- noncompliant hemodynamics, thickening of septum on Echo- “sparkling” of echoes endomyocardial biopsy shows AL deposition on immunohistochemical staining Digoxin and Nifedipine toxicity reported due to collective binding to fibrils.
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Amyloidoses Complication
AL disease - acquired deficiency of clotting factor X Periarticular amyloid deposition presenting as pseudoarthritis = shoulder pad sign
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Amyloidoses Treatment
Melphalan and Prednisone 2 controlled trials with no statiscally significant improvement in survival 20% of patients did have improvement
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Amyloidoses B2 Microglobulinemia
significant articular presentation with chronic renal disease all reported pt’s presented with joint pain carpal tunnel osteonecrosis dialysis for mean of 7 years B2 microglobulin is polypeptide of class I protein of MHC
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Amyloidoses AA Amyloid
AA deposition in tissues, associated with rheumatoid arthritis familial Mediterranean fever Recent series 5% of RA have tissue deposits and 2% have cinically evident disease, usually renal or hepatic. Worldwide, AA deposition common in leprosy, tuberculosis, osteomyelitis.
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Amyloidoses AA Amyoid Clinical - episodic fever, arthritis, abdominal/pleuritic inflammation Treatment - Colchicine abortive of acute episodes abortive of amyloid formation
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Amyloidoses Aging 5 sites identified with aging
Brain: beta protein of Alzheimer’s Pancreas: islet associated polypeptide ANF: fibril of isolated atrial amyloid Cardiac: transthyretin, senile systemic amyloid Aorta: fibril not identified
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