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31 Cards in this Set
- Front
- Back
what does SPEP do?
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detected M spike
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what does IFE do?
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identifies M spike
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what does UPEP do?
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identifies Bence Jones proteins
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what is hyperviscosity syndrome?
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huge incr in one Ig ("monoclonal protein")-->inc in serum viscosity is assoc w/visual impairment, deafness, H/A, dizziness
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cryoglobulinemia?
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when some monoclonal proteins (big incr in one Ig) precipitate at low temps
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pathogenesis of multiple myeloma
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plasma cells infiltrate tissue-->attract cytokines-->osteoclastic activity
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a bad consequence of multiple myeloma
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renal failure, caused by immunoglobulin light chain deposition & infiltration by plasma cells
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major cause of death in multiple myeloma?
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infxn
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Sx of multiple myeloma?
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lytic lesions or compression fractures
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what do you see on peripheral smear of multiple myeloma?
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rouleax formation (increased plasma protein (increased Ig)-->neutralizes the charge-->stacked RBC's)
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what does MGUS stand for?
what is the definition of MGUS? |
MGUS=Monoclonal Gammopathy of Undetermined Significance;
MGUS: -monoclonal protein< 3 g/dl (multiple myeloma: > 3g/dl) -no Bence Jones proteins -no Hypogammaglobulinemia |
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how Tx MGUS?
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no Tx
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clinical presentation of Waldenstrom's Macroglobulinemia
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hyperviscosity syndrome
cryoglobulinemia (Raynaud's phenomenon) |
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which Ig is in MGUS?
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IgM
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what do Dutcher bodies in MGUS indicate?
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neoplasm (not benign, whereas Russell bodies are benign or maligannt)
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periph blood and BM of Waldenstrom's Macroglobulinemia?
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Lymphoplasmacytoid Lymphocyte
(remember: it resembles SLL) |
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LN histology of Waldenstrom's Macroglobulinemia?
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resembles SLL
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definition of amyloid?
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protein deposited btw cells
physical nature uniform: -non-branching fibrils -beta-sheet sheet conformation |
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chemical composition of amyloid?
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1. P-component (5%)
2. Amyloid component (95%) -AL-amyloid light chain derived protein (Ig) -AA-amyloid associated protein (acute phase reactant) -transthyretin (ATTR) -beta-2 microglobulin -beta-2 amyloid protein |
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which amyloid protein is seen in multiple myeloma?
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AL
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Dx of amyloidosis
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-gross: waxy, white
-morph: amorphous, pink, hyaline -Congo red-green birefringence -physical nature is uniform: -non-branching fibrils -Beta-pleated sheet |
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primary amyloidosis
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-related to underlying plasma cell neoplasm producing abnl Ig, e.g. multiple myeloma
-abnl light chains produce AL -some pt w/monoclonal Ig-producing plasma cells do not have other manifestations of myelomas, eg no lytic bone lesions |
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reactive systemic amyloidosis
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-amyloid type=AA protein
-in pts w/underlying inflamm condition eg RA |
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hemodialysis associated amyloidosis
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Beta-2 microglobulin
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senile systemic amyloidosis
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-involes heart
-nl transthyretin (ATTR) protein deposited cf heredofamilial amyloidosis |
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wht is a plasma cell neoplasm
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increase in one, monoclonal protein-->produces M spike
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rouleax formation
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increased plasma protein (IgG), i.e. in multiple myeloma
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Tx Waldenstrom's Macroglobulinemia
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plasmapharesis (remove IgM)
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multiple myeloma has which amyloid protein?
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AL (Amyloid Light Chain derived protein (Ig))
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ppl with RA have which amyloid protein
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AA
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where do we see rouleax formation
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multiple myelomas, anemias
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