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AMYLOIDOSIS
Term | Definition |
---|---|
What is AMYLOIDOSIS? | Accumulation of extracellular deposit of Cross Beta-pleated fibrillar proteins. |
What stain is use for AMYLOIDOSIS? | Congo Red |
How does the AMYLOIDOSIS appearance? | Amorphous, eosinophilia, hyaline and extracellular substance. |
What colour occurs with polarized light, when AMYLOIDOSIS is present? | apple-green colour/birefringence |
What are the systemic or generalized types of Amyloidosis? | 1. Primary amyloidosis: --> AL (Amyloid light chain) type 2. Secondary or Reactive systemic amyloidosis --> AA (amyloid associated) type 3. Hemodialysis-associated amyloidosis |
What type of tumor is Primary amyloidosis associated with? | Plasma cell Tumors --> Multiple myeloma and B- lymphoproliferative diseases like B-cell lymphoma, non-hodgkin lymphoma and Waldenstrom's Macroglobulinemia. |
What is the cause of primary amyloidosis? | Clonal expansion of the bone marrow plasma cells --> Immunoglobulin light chains, that is, kappa/lambda light chains. |
What is the cause of secondary/reactive amyloidosis? | Elevated level of acute-phase protein --> leads to the increase serum amyloid A proteins |
What cause the elevated level of acute-phase protein? | Increase levels of IL-1 and IL-6. |
What cause the body to be in an inflammatory state during secondary amyloidosis? | SAA--> Serum Amyloid A protein |
What is secondary amyloidosis initially associated with regards to organs? | Kidneys. Results in proteinuria followed by progressive renal dysfunction--> nephrotic syndrome. |
What is the cause of Hemodialysis-associated amyloidosis? | Long term hemodialysis/ End stage renal disease --> leads to elevated Beta 2 - microglobulin |
Where does amyloid deposit (Hemodialysis-associated amyloidosis)? | synovial joint or bones Cause carpal tunnel syndrome. |