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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
-no Hypogammaglobulinemia
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:
-non-branching fibrils
-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)
-transthyretin (ATTR)
-beta-2 microglobulin
-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:
-non-branching fibrils
-Beta-pleated sheet
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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