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28 Cards in this Set
- Front
- Back
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What IS amyloid NOT?
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It's NOT just a single substance
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What IS amyloid?
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A GROUP of substances that share a common physical structure formed by a variety of different proteins.
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What is the underlying feature of the Amyloid that makes all these substances similar?
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B-pleated sheet configuration
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Why is this B-pleated sheet configuration important?
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It causes the proteins to accumulate and form deposits.
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Where are the deposits formed in amyloidoses?
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EXTRACellUlArlY - often right next to the basement membrane.
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What characteristic STAIN will be positive in amloidosis?
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CONGO RED dye
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What confirms the diagnosis of Amyloidosis after detection with the Congo red dye?
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Demonstration of apple green BIREFRINGENCE under polarized light
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What are the 3 main clinical patterns of amyloidosis?
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-Primary / immunocytic dyscrasia
-Secondary / Reactive systemic -Other forms |
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What are the amyloid fibrils that cause Primary amyloidosis derived from?
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Immunoglobulin light chains (either K/L) - AL protein
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With what clinical diagnoses is AL protein most commonly seen?
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Plasma cell dyscrasias:
-Waldenstrom's macroglobulinemia or -Multiple myeloma |
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In what tissues is AL protein usually deposited?
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Mesenchymal
-Heart -Lung -Tongue sometimes Kidney |
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What are the amyloid protein fibrils in Secondary reactive amyloidosis derived from?
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Serum Amyloid Associated protein (SAA) - a precursor
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What are the characteristic sites of tissue deposition of the AA protein in secondary amyloidoses?
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PARENCHYMATOUS organs
-Liver -Adrenals -Pancreas -Spleen/Lymph nodes |
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In what diseases is 2ndary amyloidosis characteristically a complication?
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Chronic Inflammatory Diseases:
-RA -TB/Leprosy -Syphilis -Osteomyelitis |
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What is Portuguese type amyloidosis? Symptoms?
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Deposition of TRANSTHYRETIN derived amyloid deposits
Severe polyneuropathy |
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What is the normal function of Transthyretin?
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Transport of RETINol and THYRoid hormone
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What type of amyloid protein is seen in Alzheimer's?
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A-Beta
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What is the gene that encodes for the precursor proteins for AB-amyloid?
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Ch' 21
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Cancer associated with prominent amyloid deposits inside it:
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MCT - medullary thyroid carcinoma
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What cell type is MTC derived from?
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C-cells of the parafollicular spaces - Calcitonin cells
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Where are amyloid deposits found in some patients with DM type II?
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In the ISLET cells
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What is the amyloid of type II DM called?
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Amylin or Islet amyloid polypeptide
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What is the amylin deposited in islets in DM2 thought to be a contributing cause of?
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Insulin insensitivity of the beta cells
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In what condition are Amyloid B2M deposits found in the joints of patients?
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Patients on chronic dialysis for ESRD
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What is the protein that the amyloid is derived from in chronic dialysis? Why does it accumulate?
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B2-microglobulin - It's not filtered readily by the dialysis membrane.
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So AL-Amyloidosis is:
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Light chain immunoglobulin - Plasma cell dyscrasias
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And the hallmark stain for Amyloid is:
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CONGO RED
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In senile amyloidosis found at autopsy, if deposits are seen in the heart they are probably:
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Transthyretin derived
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