Flashcards in Ch 23: Amyloidoses Q & A Deck (14):
Diagnosis of amyloidosis requires what for confimation?
All forms of amyloid stain (+) with this & show red-green birefringence when viewed under polarized light.
Presentation of amyloidosis w/o any precceeding disease. Consists of variable region of immunoglobulin light chains & can be derived from either kappa or lamda polypeptides.
AL Amyloidosis (Primary)
Occurs in patients w/ seemingly unrelated, persistant, inflammatory or neoplastic disorders. Ex. A patient w/ long standing RA, complains of swelling of eyelids, abdomen & ankles. Increase of what protein during inflammation causes what disease? What is the amyloidogenic form of this protein?
AA Amyloidosis (Secondary)
Serum Amyloid A to Apo Serum Amyloid A (amyloidogenic)
Amyloidosis refers to a group of extracellular protein deposits that are best defined as what?
Proteins with a common morphologic properties
Progessive cell & organ dysfunction will develop primarily as a result of what biological process in AA/AL amyloidosis?
Diminished blood supply to parenchymal cells.
Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant disorder that is associated with a mutation in a gene for what protein?
This disorder usually manifests as paresthesias, w/ loss of temperature & pain sensation.
This is found in a woman with progressive neurodegenerative disease. Autopsy reveals spongiform encephalitis with brain amyloidosis
APrP (Amyloid Prion Protein)
Derived from the protein observed in a congo red stain from a patient suffering from cognitive impairment. This protein gets deposited in the brain and cerebral vessels.
Patients with Down syndrome are associated with Alzheimer's disease. Autopsy would reveal cerebral deposits of what protein?
A patient presents with hypoalbuminemia, mild anemia & thrombocytopenia. Monoclonal peak is demonstrated by serum gel electrophoresis & a bone marrow aspiration demonstrates numerous atypical plasam cells. Proteinuria, polyuria & polydipsia on presentation. Renal biopsy would show deposits of what amyloid precursor proteins?
Immunoglobulin Light Chain
Medullary carcinoma of the thyroid originates from C cells (APUD Cell) of the thyroid. The amyloid deposits are going to be most closely related to what serum protein?
Deposition of this protein in major joints of patients undergoing chronic renal dialysis is characterized by a destructive arthopathy