2nd Exam: Plasma Cell Disorders Flashcards Preview

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Flashcards in 2nd Exam: Plasma Cell Disorders Deck (175)
1

CD 3, 4, 5:

T lymphocytes

2

CD 13:

most granulocytees

3

CD15:

granulocyes

4

List the 5 heavy chains:

GAMED

5

Common Ig's?

IgG: most common, A and M: next most common, D and E: fairly uncommon

6

1 plasma cell, 1:

Ig (one type of each chain)

7

3 classifications o plasma cell disorders:

MGUS, plasma cell myeloma Plasmsmacytoma

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MGUS:

Monoclonal gammopathy of undertermined significance

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Solitary lesion of neoplastic monoclonal plasma cells morphologically the same as myeloma, bone or soft tissue:

plasmacytoma

10

Related Ig producing disorders:

Ig depositiondisease, ie primary amyloidosis, Heavy chain disease, adn lymphoplasmacytoid Lymphoma

11

progressive, incurable, excess light chains bind, producing amyloidosis: abnormal protein deposition in one or more organs or body systems.

primary amyloidosis

12

Heavy chains produced in Heavy Chain Disease:

G, A, or M, no light chains

13

Lymphoplasmacytoid Lymphoma

Subset IgM producing: Waldenstrom's Macroglobulinemia

14

Waldenstrom's Macroglobulinemia is what type of disorder:

Lymphoplasmacytoid lymphoma

15

Disorder of malig neo of lympholyctes stuck at lympho and plasma cell differentiation border:

Lymphoplasmacytoid Lymphoma

16

Antigen binding-sites on Ig's are made of:

amino ends (NH2)

17

kappa/lambda light chain ration in peripheral blood:

2:1

18

Digests IgG:

papain

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IgG is broken into these via papain:

2 identical FAB, 1 Fc

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FAB sf:

Fragment antigen binding

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Fc sf:

fragment-crystallizable (constant end of Ig)

22

Major Ig in serum:

G

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Major Ig of external secretions:

A

24

1st Ig presesnt after primary antigenic stimulus:

M

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Ig that crosses placenta and Ig that does not.

G, M

26

Ig in trace amts in normal serum:

D

27

define reaginin:

allergic

28

This has reaginic antibody activity

IgE

29

IgE is involved in:

immediate hypersensititvity, eosinophilic response

30

IgG crosses placenta via:

active transport

31

When is IgG produced:?

response to 2nd exposure to an antigenic stimulus

32

Major Ig in GI secretions:

A

33

Ig considered large size "macrolobulin:

M, polymer/ pentamer

34

1st Ab produced in infectious mononucleosis:

M

35

infectious mononucleosis develops from:

EBV esp. college age

36

Test for these Ig's if you suspect infectious mononucleosis:

M, G, and A

37

Ig levels that rise in response to EBV virus:

M and A

38

Ig's levels increased with recent exposure to EBV, recent mono infection:

lots of M, no G

39

Test for mono, IgG is high and IgM is not:

had mono in past, not current infection

40

Order of Ig presentation in mono infection:

M then G, stays high for a while later

41

If Rafi were tested now for mono, which Ig would be present in higher conc's?

G

42

Ig produced to fight issues in GI tract:

A

43

EBV is aka:

HHV-4

44

Swollen lymph nodes and suspected mono indicates:

G could be high or not, mono in past

45

Slide appearance of plasma cells when they are mounting a response:

making lots of Ig, bluish cytoplasm, eccentric nucleus

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Polyclonal proliferation of plasma cells that are each different, many types of Ig, some kappa, some lambda:

polyclonal plasma cell proliferation

47

Plasma cells proliferate in response to:

antigen presentation by B lymphos, many lines searching for best Ig to produce

48

polyclonal plasma cell proliferation, neoplastic or malignant cell line?

either

49

neoplastic or malignant cell line, one or many cells of origin?

1, same products, 1 light chain, 1 Ig type, 1 marker

50

Leads to a monoclonal serum protein or "M" protein:

production of a monoclonal light chain by neoplastic plasma cells or B-lymphos

51

Stain this for monoclonal plasma cells:

either light chain

52

SPEP sf:

serum protein electrophoresis, agarose gel electrophoresis to id different proteins in serum via size and electrical charge

53

Clinical syms pointing toward monoclonal gammopathy:

abnormal SPEP, intermittent, "non-sp" joint pain, no swelling or redness

54

Test to use when a monoclonal serum protein is suspected:

SPEP

55

Normal SPEP has, abnormal has:

5 regions, goal post spikes at either end/ gamma region: albumin on L, Gamma globulin on R

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List the 5 regions in normal SPEP:

serum albumin, alpha-1, alpha 2, beta, gamma globulins

57

Spike on normal SPEP with 2 hills:

beta

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What spike would be down in SPEP w nutritional problems?

albumin region spike

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What does a broad, increased band in gamma region indicate?

fighting infection, probably polyclonal

60

Test to measure kidney function:

BUN/ Creatinine

61

Normal % of plasma cells in bone marrow:

less than 5%

62

What test next with slight increase in monoclonal protein in blood, all else WNL, spike in y-region of SPEP:

IFE: Immunofixation electrophoreses

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IFE sf:

Immunofixation electrophoreses

64

When to do IFE:

to further evaluate SPEP findings, id and characterize monoclonal proteins in serum or urine

65

In IFE, after proteins from urine or serum are separated they are incubated with:

antisera against Ig of interest, precipitates at sites of Ag-Ab reaction

66

Band intesity of IFE correlates with:

protein concentration

67

Tx for MGUS:

none, asymptomatic, simply follow

68

monoclonal serum protein is aka:

"M" protein

69

TF? Presence of an expanded clone of Ig-secreting cells is considered neoplastic.

F, does not always progress to overt malignancy

70

What is present in serum with MGUS?

small amt monoclonal serum protein ("M" protein", <3gm/dl), bm plasma cells <10%

71

Most common cause of monoclonal gammopathy:

MGUS

72

MGUS common in this pop:

elderly: 3% over 50yo, 5% over 70yo

73

Symptoms of MGUS:

none

74

MGUS may evolve to become:

well defined palsma cell or lymphoid neolpasm

75

Risk of MGUS progression per year:

1.6%, increased likelihood of having it every year

76

Clinical symps of plasmacytoma:

peristant pain in discreet region, osteolytic lesion on x-ray, elevated IgG, IgA and IgM: low normal, low % of plasma cells in bm, sheet of monoclonal plasma cell, high density (1 light chain type) in biopsy

77

Tx of plasmacytoma:

focal radiation therapy

78

Plasmacytoma has similar clinical presentation as:

myeloma, but no lytic bone lesions, normal bm biopsy

79

Name of plasmacytoma found in bone:

"solitary plasmacytoma of bone"

80

Name of plasmacytoma found in soft tissue:

extra-osseous plasmacytoma

81

Most common site of solitary plasmacytoma of bone

Spine/vertebra

82

Fraction of plasmacytomas that progress to myeloma or additional plasmacytomas:

up to 2/3

83

Initial symptoms of plasmacytoma :

swelling or bone pain, lesion on xr

84

Clinical sym of plasma cell myeloma:

dizziness, light headed, inc bone pain, IgG increased, decreased IgA and IgM, generalized osteoporosis, lytic lesions/ dec bone mass, impaired renal function (abnormal BUN/creatinine), bm packed with monoclonal plasma cells, many abnormally shaped

85

TF? There is a finite level to the amt of Ig your body can produce.

T. One level goes up, another goes down

86

Plasma cell myeloma is aka:

multiple myeloma

87

Abnormal SPEP is found in:

monoclonal gammopathy and multiple/ plasma cell myeloma

88

Difference bw SPEP of monoclonal gammopathy and multiple/ plasma cell myeloma:

monoclonal: posts about same height, multiple: y-region taller than albumin region

89

Clinical dx of symptomatic multiple myeloma:

M-protein in serum or urine, bm clonal plasma cells or plasmacytoma, related organ tissue impairment: CRAB, suppression of normal Ig's leading to infection, excessive IgG or IgA (kidney damage)

90

CRAB sf:

Hypercalcemia, renal insufficiency, anemia, bone lesions

91

Clinical problems pts with multiple myeloma may encounter:

CRAB

92

Clinical dx of asymptomatic multiple myeloma:

M protein in serum/urine (<3% g/dl) AND/OR 10% or more clonal plasma cells in bm marrow BUT no related organ or tissue impairment (no CRAB) or myeloma related symptoms

93

asymptomatic multiple myeloma is aka:

smoldering plasma cell myeloma

94

If a young person got MM (rare) this procedure would probably be done:

bm transplant

95

Median age for MM onset:

70yo, 1% US cancer deather

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Most common plasma cell dyscrasia:

MM

97

Tx for MM:

chemo, radiation for sp lesions (palliation), bm transplant

98

define palliate:

make less unpleasant wo removing cause:

99

Hypercalcemia seen in MM is due to:

bone destruciton

100

Inceased levels of these Ig's can lead to kidney damage:

G, A

101

Suppression of normal Ig's leads to:

infection

102

This presents as "punched out" bone:

MM

103

What to suspect if a pt breaks a bone during normal life activities:

MM, underlying bone destruction

104

Hypercalcemia leads to:

confusion, weaknesss, lethargy, deposits

105

Why does anemia present as a symptom of MM?

bm infiltration

106

Can MM involve organs besides bone and soft tissues?

ys

107

Most common site of lytic bone lesions in MM:

vertebrae

108

somnolent:

sleepy, drowsy

109

Lab results with MM:

inc blood Ca2+ CPR, B2-microglobulin, anemia, monoclonal protein id'ed using SPEP and IFE

110

B2-microglobulin

component of MHC class I mol's, on all nucleated cells

111

Why to take multiple xr's with MM:

punched out appearance is not present throughout entire body

112

Lesions of vertebrae with red marrow, juicy, cellular, many abnormal plasma cells, rounded lesions suspect this:

MM

113

Disorder that can lead to collapsed vertebrae:

MM

114

Do normal plasma cells have nucleoli?

no

115

Ig inclusions are called:

Russell bodies

116

Russel bodies are seen in:

normal plasma cells and myeloma cells, pts with M spike

117

cells with many Ig inclusions:

Mott cells, pts with m spike

118

TF? The presence of mott cells indicates malignancy.

F.

119

Found in the vacuoles of mott cells and russell bodes:

Ig

120

Tx to reverse spinal cord compression due to MM:

emergency bm biopsy, chemo

121

BM findings with MM:

increase plasma cells, often abnormal, variable disease distribution in bm

122

Peripheral blood findings with MM:

anemia w or wo dec wbcs, thrombocytopenia (dec platelets), maybe plasma cells, stacking of rbcs (rouleaux) due to inc serum protein, maybe plasmacytoid lymphos (increased calcium, right? CRAB?)

123

Cause of roleaux:

inc serum protein

124

What is roleaux?

rbc stacking due to inc protein in blood

125

Why is the background bluish when staining of MM peripheral blood:

protein staining

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To test for amyloid

Congo red stain

127

amyloid due to MM can cause

enlarged tongue

128

leading causes of death with MM:

#1: infections, #2: kidney failure

129

Cause of kidney damage with MM:

monoclonal light chains form tubular casts, toxic to tubular epi cells, plasma infiltration, inc Ca2+ levels due to renal vasoc, leading to intra-tubular Ca+2 deposits, infection, amyloid deposition (how are IgG and A involved?)

130

monoclonal light chains in urine:

Bence Jones proteins

131

Kindey can be damaged by hypercalceremia due to:

renal vasoc, leads to intra-tubular calcium deposition

132

use dipstick urine test to detect:

only albumin, not immunoglobulin

133

to detect monoclonal light chains in urine:

urine protein electrophoresis

134

Tests to order of pts with MM and kidney issues:

SPEP and immunohistochemistry

135

How do light chains damage kidney tubules?

solidify to form light chain casts, cast nephropathy

136

formation of plugs in renal tubules from free Ig light chains leading to renal failure in context of MM:

cast nephropathy

137

Where does Ca2+ precipitate in body?

random spots, including kidney

138

Myeloma staging is based on:

bone lesions, anemia, inc blood Ca2+, creatinine, Ig levels, Bence-Jones proteins in urine

139

Creatinine measures are related to:

kidney function

140

Hct sf:

hematocrit

141

Tx for myeloma:

radiation, chemo, stem cell transplant, velcade, Interferon-y (anti-IL-6 agent), interferon-a, thalidomide, bisphosponates

142

Velcade is aka:

bortezomib

143

Anti-IL6-agent:

interferon-y

144

Function of bisphosphonates:

inhibit bone resorption, reduce osteoclasts

145

Drugs used for chemo tx of MM:

melphalan, cyclophosphamide, prednisone

146

Side effect of chem tx for MM:

myelosuppression

147

Velcade (bortezomib):

proteasome inhibitor, inc myeloma cell response to chemo, induce apoptosis of tumor cells, side effect: neuropathy

148

Drugs used to tx MM besides chemo:

velcade (bortezomib), anti-Il-6 agents (iterferon-y), interferon-a,m thalidomide, bisphosphonates

149

We want to decrease IL-6 in MM tx because:

IL-6 promotes myeloma cell proliferation

150

Function of interferon-a:

turns off osteoclasts, aimed at bone lesions (how do we ensure only the lesion osteoclasts are turned off?)

151

Functions of thalidomide:

anti-TNFa-immunomodulating, inhibits angiogensis

152

Pink stained kidney indicates:

amyloid in kidney, light chain deposition, deproteinaseous material

153

Test to detect amyloid in kidney:

Congo red staining, polarized light, apple green

154

Plasma cell disorder with increased amyloid levels (amyloidosis):

any

155

Fibrillary protein deposited in various tissues, may damage organs:

amyloid

156

plasma cell neoplasm, amyloid derived from all or part of monoclonal Ig light chain secreted by plasma cells:

primary amyloidosis

157

TF? Amyloidosis is always related to plasma cell disorders.

F, can be seen by itself

158

These combine to form amyloid fibrils:

protofilaments

159

Test to id amyloid fibers:

congo red, apple green birefringence under polarized light or EM

160

Risk factors for primary amyloidosis:

60-70yo, male (2/3)

161

How to dx primary amyloidosis:

demonstration of amyloid in biopsy specimen (Congo red stain)

162

Test to id the type of amyloid in 1' amyloidosis:

mass spec

163

Oral manifestations of amyloidosis:

enlarged tongue, periorbital bleeding, should biopsy

164

Waldenstrom's macroglobulinemia:

IgM secretions, monoclonal protein (macroglobulin), diffuse infiltrate of neoplastic B-cells (small lymphos with plasmacytoid features) in bm, lymph nodes, liver and spleen

165

Median survival of Waldenstrom's macroglobulinemia:

4y

166

Clinical presentation of Waldenstrom's macroglobulinemia:

weaknes, fatigue, w8 loss, lymphadenopathy, large liver or spleen, hyperviscocity syndrome, typically 50-60yo

167

hyperviscocity syndrome:

visual and nerve problems, bleeding (acquired factor deficiencies, cryoglobulinemia, proteins insoluble at temps below body)

168

macroglobulin Ig:

IgM

169

Ig precipitation at cold temperatures, in fingers is indicative of:

Waldenstrom's macroglobulinemia

170

Hyperviscocity in Waldenstrom's macroglobulinemia is due to:

Inc IgM production, neoplastic production, can't get through caps, think of eyes, bleeding, absorb clotting factors

171

Tx for blood build up in eyes due to Waldenstrom's macroglobulinemia:

plasmaphoresis to remove protein: blood run through machine, prevent retina destruction

172

Gelling or precipitation of a monoclonal protein at low temp:

cryoglobulin

173

Ig most often to become a cryglobulin:

IgM

174

Clinical presentation of cryoglobulinemia:

sluggish blood flow, purpura, Reynaud's phenomenon, gangrene

175

artery spasm, dec blood, typically fingers, sometimes toes, rarely nose, ears, lips, turns white, then blue, numb, painful, red and burning when blood flow returns, Recurrent attacks - brittle nails w longitudinal ridges:

Reynaud's phenom