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