Path-Heme-Plasma Cell Neoplasms

Front 1

what does SPEP do?

Back 1

detected M spike

Front 2

what does IFE do?

Back 2

identifies M spike

Front 3

what does UPEP do?

Back 3

identifies Bence Jones proteins

Front 4

what is hyperviscosity syndrome?

Back 4

huge incr in one Ig ("monoclonal protein")-->inc in serum viscosity is assoc w/visual impairment, deafness, H/A, dizziness

Front 5

cryoglobulinemia?

Back 5

when some monoclonal proteins (big incr in one Ig) precipitate at low temps

Front 6

pathogenesis of multiple myeloma

Back 6

plasma cells infiltrate tissue-->attract cytokines-->osteoclastic activity

Front 7

a bad consequence of multiple myeloma

Back 7

renal failure, caused by immunoglobulin light chain deposition & infiltration by plasma cells

Front 8

major cause of death in multiple myeloma?

Back 8

infxn

Front 9

Sx of multiple myeloma?

Back 9

lytic lesions or compression fractures

Front 10

what do you see on peripheral smear of multiple myeloma?

Back 10

rouleax formation (increased plasma protein (increased Ig)-->neutralizes the charge-->stacked RBC's)

Front 11

what does MGUS stand for?
what is the definition of MGUS?

Back 11

MGUS=Monoclonal Gammopathy of Undetermined Significance;

MGUS:
-monoclonal protein< 3 g/dl (multiple myeloma: > 3g/dl)
-no Bence Jones proteins
-no Hypogammaglobulinemia

Front 12

how Tx MGUS?

Back 12

no Tx

Front 13

clinical presentation of Waldenstrom's Macroglobulinemia

Back 13

hyperviscosity syndrome
cryoglobulinemia (Raynaud's phenomenon)

Front 14

which Ig is in MGUS?

Back 14

IgM

Front 15

what do Dutcher bodies in MGUS indicate?

Back 15

neoplasm (not benign, whereas Russell bodies are benign or maligannt)

Front 16

periph blood and BM of Waldenstrom's Macroglobulinemia?

Back 16

Lymphoplasmacytoid Lymphocyte

(remember: it resembles SLL)

Front 17

LN histology of Waldenstrom's Macroglobulinemia?

Back 17

resembles SLL

Front 18

definition of amyloid?

Back 18

protein deposited btw cells
physical nature uniform:
-non-branching fibrils
-beta-sheet sheet conformation

Front 19

chemical composition of amyloid?

Back 19

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

Front 20

which amyloid protein is seen in multiple myeloma?

Back 20

AL

Front 21

Dx of amyloidosis

Back 21

-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

Back 22

-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

Front 23

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

Front 25

senile systemic amyloidosis

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-involes heart
-nl transthyretin (ATTR) protein deposited cf heredofamilial amyloidosis

Front 26

wht is a plasma cell neoplasm

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increase in one, monoclonal protein-->produces M spike

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rouleax formation

Back 27

increased plasma protein (IgG), i.e. in multiple myeloma

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Tx Waldenstrom's Macroglobulinemia

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plasmapharesis (remove IgM)

Front 29

multiple myeloma has which amyloid protein?

Back 29

AL (Amyloid Light Chain derived protein (Ig))

Front 30

ppl with RA have which amyloid protein

Back 30

AA

Front 31

where do we see rouleax formation

Back 31

multiple myelomas, anemias