MIDTERM LECTURE 2: ACUTE MYELOID LEUKEMIA Flashcards

1
Q

most common type of leukemia in adults

A

AML

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2
Q

FAB classification is identified based on

A

morphology and cytochemistry

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3
Q

WHO classification is identified based on

A

molecular characterization and cytogenetics

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4
Q

General clinical presentation of AML

A

1) decrease production of normal BM elements
2) presence of myeloblast
3) WBC count: 5-30x10^9/L
4) bleeding abnormalities
5) infiltration of malignant cells into the gums and other mucosal sites
6) CNS related symptoms

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5
Q

wbc count of px w/ AML

A

5-30x10^9/L

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6
Q

bleeding abnormalities in AML is associated with what disorder

A

disseminated intravascular coagulation (DIC)

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7
Q

clinical findings in AML

A

1) anemia
2) thrombocytopenia
3) neutropenia
4) pallor
5) fatigue
6) fever
7) bruising
8) bleeding
9) splenomegaly

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8
Q

anemia in AML is due to

A

overproduction of blast cells

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9
Q

neutropenia in AML is due to

A

blast cells tend to forget to mature

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10
Q

how many percentage of px w/ can be seen with splenomegaly

A

50%

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11
Q

abnormalities in laboratory results in AML

A

1) hyperuricemia
2) hyperphosphatemia
3) hypocalcemia
4) hypokalemia

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12
Q

cause of hyperuricemia in AML

A

caused by increased cellular turnover

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13
Q

cause of hyperphosphatemia

A

due to cell lysis

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14
Q

cause of hypocalcemia

A

hyperuricemia and hyperphosphatemia involves in bone destruction

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15
Q

group of metabolic complications that occurs in px with malignancy, with or without treatment

A

tumor lysis syndrome

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16
Q

tumor lysis syndrome is notable in what diseases

A

lymphoma and leukemia

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17
Q

tumor lysis syndrome is caused by

A

breakdown of the products of dying cancer cells

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18
Q

how does tumor lysis syndrome lead to renal failure

A

dying cancer cells products-> acute uric acid nephropathy-> renal failure

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19
Q

tumor lysis syndrome is characterized by

A

hyperkalemia
hyperphosphatemia
hyperuricemia
hyperuricosuria

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20
Q

subtypes of AML according to WHO classification

A

1) AML w/ recurrent genetic abnormalities
2) AML w/ myelodysplasia-related changes
3) therapy-related myeloid neoplasms (t-MNS)
4) AML, not otherwise specified
5) Myeloid Sarcoma
6) Myeloid Proliferations Related to Down Syndrome
7) Blastic Plasmacytoid Dendritic Cell Neoplasm
8) Acute Leukemias of Ambiguous Lineage

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21
Q

AML with recurrent genetic abnormalities

A

1) Acute myeloid leukemia with t(8;21)(q22;q22.1); RUNX1/RUNX1T1
2) AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFV-MYH11
3) Acute promyelocytic leukemia with PML-RARA
4) AML with t(9;11)(p22;q23); KMT21A (MLL)-MILLT3
5) AML with t(6;9)(p23;q34.1); DEK-NUP214
6) AML with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM (RPN1-EVI1)
7) AML with t(1;22)(p13.3;q13.3) RBM15-MKL1
8) AML with BCR-AML1
9) AML with gene mutations
10) AML with mutated NPM1
11) AML with biallelic mutation of CEBPA
12) AML with mutated RUNX1

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22
Q

identify the mutation:
AML with t(8;21)(q22;q22.1)

A

RUNX1-RUNX1T1

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23
Q

identify the mutation:
AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22)

A

CBFB-MYH11

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24
Q

identify the mutation:
AML with t(9;11)(p22;q23)

A

KMT21A (MLL)-MLLT3

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25
Q

identify the mutation:
AML with t(6;9)(p23;q34.1)

A

DEK-NUP214

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26
Q

identify the mutation:
AML with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2)

A

GATA2, MECOM (RPN1-EVI1)

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27
Q

identify the mutation:
AML with t(1;22)(p13.3;q13.3)

A

RBM15-MKL1

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28
Q

identify the AML:
-seen predominantly in children and young adults
-prognosis is favorable but may be negatively affected due to addition of abnormalities

A

RUN X1/RUNX1T1

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29
Q

AML RUNX1/RUNX1TI is found in how many percent of AML cases

A

5%

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30
Q

RUNX1/RUNX1T1 is diagnosed based on

A

genetic abnormality

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31
Q

RUNX1/RUNX1T1 clinical findings

A

-myeloblast w/ dysplastic cytoplasm
-auer rods

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32
Q

RUNX1/RUNX1T1 anomalies

A

-pseudo-Pelger-Huet cells
-hypogranulation
-eosinophilia

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33
Q

CBDB-MYH11 is also classified as what AML

A

Core-binding factors (CBF) AML

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34
Q

AML CBFB-MYH11 accounts for how many percentage of AML cases

A

5-8%

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35
Q

age of px that can develop Core-binding factors (CBF) AML

A

all ages

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36
Q

CBFB-MYH11 is predominant in what age

A

younger patients

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37
Q

diagnosis for CBFB-MYH11

A

genetic aberration

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38
Q

CBFB-MYH11 increases the incidence of what disease

A

extramedullary disease

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39
Q

common site for relapse in CBFB-MYH11

A

central nervous system

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40
Q

T or F:
remission is good in CBFB-MYH11

A

T
(but only 1/2 are cured)

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41
Q

identify the AML:
-characterized by differentiation block at the promyelocytic stage

A

APL w/ PML-RARA

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42
Q

percentage of PML-RARA in AML cases

A

5-10%

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43
Q

what age is PML-RARA predominant

A

young adults

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44
Q

diagnosis for PML-RARA

A

15;17 translocation

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45
Q

subtype of APL that accounts for 13-40% of APL cases and gives an appearance of having no granules

A

microgranular variant

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46
Q

how to differentiate APL with AML

A

1) auer rods
2) butterly/coin-on-coin nucleus
3) clinical presentation

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47
Q

treatment for APL w/ PML-RARA

A

1) All-trans-retinoic acid (ATRA)
2) Arsenic trioxide

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48
Q

what treatment of APL w/ PML-RARA is a vitamin A analogue and induces differentiation of malignant promyelocytes

A

ATRA

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49
Q

identify the AML:
-represents AML subgroups w/ 11q23 abnormalities

A

KMT2A (MLL)-MLLT3

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50
Q

what AML translocation is rare

A

AML with t(9;12)

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51
Q

characteristics of KMT2A (MLL)-MLLT3

A

1) large blast w/ abundant cytoplasm
2) fine nuclear chromatin
3) motile cells w/ pseudopodia

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52
Q

T or F:
KMT2A (MLL)-MLLT3 is frequently seen in adults

A

F
(frequent in children)

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53
Q

KMT2A (MLL)-MLLT3 is associated with what conditions

A

associated with gingival and skin involvement and DIC

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54
Q

identify the AML:
-20% blast cells
-multilineage dysplasia
-history of MDS/MPL
-MDS-associated cytogenetic abnormality

A

AML w/ myelodysplasia-related changes

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55
Q

AML w/ myelodysplasia-related changes should have the absence of what AML

A

AML w/ recurrent genetic abnormalities

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56
Q

AML w/ myelodysplasia-related changes primarily affects what age group

A

older adults

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57
Q

morphologic criteria for multilineage dysplasia

A

50% dysplasia in 2 lineages

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58
Q

significant dysplastic morphology of AML w/ myelodysplasia-related changes

A

1) pancytopenia w/ neutrophil hypo/hypergranulation
2) pseudo-Pelger-Huet cells
3) unsually segmented nuclei

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59
Q

describe the erythrocyte precursors of AML w/ myelodysplasia-related changes

A

1) vacuolated
2) karyorrhexis
3) megaloblastoid features
4) ring sideroblast

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60
Q

genetic findings of AML w/ myelodysplasia-related changes

A

complex karyotypes, -7/del(9q), and 5/del(5q)

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61
Q

identify the AML:
-accounts for 10-20% of AMLs, MDSs, and MDSs/MPNs
-occurs secondary to treatment/malignancy

A

AML w/ therapy-related myeloid neoplasm (T-MNs)

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62
Q

Classifications of AML w/ TMNS

A

1) therapy-related MDS
2) AML (t-AML)
3) myelodysplastic/myeloproliferative neoplasms (t-MDS/MPN)

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63
Q

contributors for developing AML w/ T-MNs

A

1) alkylating agents
2) radiation
3) topoisomerase II

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64
Q

prognosis of AML w/ T-MNs

A

poor

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65
Q

what therapy-related neoplasm mutation behave more like the de novo counterparts

A

t(15;17) and inv(16)

66
Q

identify the AML:
-occurrence of extramedullary proliferation of blast of one or more lineages
-disrupts the tissue architectures

A

myeloid sarcoma

67
Q

commonly affected tissues in myeloid sarcoma

A

1) skin
2) GI tract
3) lymph nodes

68
Q

10% of newborns that are present with abnormal myelopoiesis have what genetic abnormality

A

trisomy 21

69
Q

identify the AML:
-spontaneous remission occurs within few months
-associated with GATA1 mutations

A

Myeloid proliferations related to down syndrome

70
Q

Myeloid proliferations related to down syndrome has AML during the first 5 years of life increase by how much

71
Q

leukemia lineage of myeloid proliferations related to down syndrome

A

megakaryocytic lineage

72
Q

treatment for myeloid proliferations related to down syndrome

A

chemotherapy

73
Q

prognosis of myeloid proliferations related to down syndrome

A

young children: responds well
older children: do not fare

74
Q

identify the AML:
-rare clinically aggressive tumor
-present skin lesions
-may progress involving peripheral blood and BM

A

Blastic Plasmacytoid Dendritic Cell Neoplasm

75
Q

Blastic Plasmacytoid Dendritic Cell Neoplasm is derived from what precursors

A

plasmacytoid dendritic cell

76
Q

AML category that do not fit into WHO subtypes

A

AML, not otherwise specified

77
Q

WHO classification of AML not otherwise specified is identified according to

A

1) morphology
2) cytometric phenotypic
3) limited cytochemical reactions

78
Q

FAB classification of AML not otherwise specified is identified according to

A

1) cell origin
2) degree of maturity
3) cytochemical reactions
4) limited cytogenetic features

79
Q

AML, not otherwise specified requires how many percentage of blast to be diagnosed

80
Q

AML, not otherwise specified accounts for how many percent of AML cases

81
Q

FAB classification of AML with minimal differentiation

82
Q

blast description of AML with minimal differentiation

A

CD13+, CD33+, CD34+, CD117+

83
Q

clinical presentation of AML with minimal differentiation

A

1) auer rods are absent
2) no clear evidence of cellular maturation

84
Q

AML with minimal differentiation accounts how many percent of AML cases

85
Q

T or F:
AML with minimal differentiation is predominant in either infants or adults

86
Q

cytochemical stain results of AML with minimal differentiation

A

1) MPO +
2) SBB +
3) NASDA +
4) ANAE -
5) ANBE -

87
Q

FAB classification of AML without maturation

88
Q

blast description of AML without maturation

A

CD13+, CD33+
majority of the cases: CD117+, CD34

89
Q

clinical presentation of AML without maturation

A

1) blasts comprise 90% of nonerythoid cells
2) <10% of leukocytes show maturation to the promyelocyte stage or beyond
3) at least 3% of blasts give positive results with MPO and SBB

90
Q

cytochemical stain results of acute myeloid leukemia without maturation

A

1) MPO +
2) SBB +
3) NASDA +
4) ANAE -

91
Q

FAB classification of AML with maturation

92
Q

blast description of AML with maturation

A

1) >20% blast
2) at least 10% maturing cells of neutrophil lineage
3) <20% precursors with monocytic lineage

93
Q

clinical presentation of AML with maturation

A

auer rods are often present

94
Q

cytochemical stain results of acute myeloid leukemia with maturation

A

1) MPO +
2) SBB +
3) NASDA +
4) ANAE -
5) ANBE -

95
Q

FAB classification of Acute myelomonocytic leukemia

96
Q

blast description of acute myelomonocytic leukemia

A

1) has myeloid and monocytoid cells in peripheral blood and BM
2) 20% monocytic cell, neutrophils, precursors
3) monoblast are large w/ abundant cytoplasm
4) cytoplasm w/ small granules and pseudopodia
5) nucleus is large and immature, has contorted nuceloli

97
Q

(+) myeloid antigens in M4

A

CD13, CD33

98
Q

(+) monocytic antigens in M4

A

CD14,CD4,CD11b, CD11c, CD64

99
Q

clinical presentation of AML with maturation

A

elevated WBC count

100
Q

cytochemical staining results of acute myelomonocytic leukemia

A

1) MPO +
2) SBB +
3) NASDA +
4) ANAE +
5) ANBE +

101
Q

FAB classification of Acute monoblastic and monocytic leukemias

102
Q

Acute monoblastic and monocytic leukemias is aka

A

Schilling’s leukemia

103
Q

80% of cells in Acute monoblastic and monocytic leukemia are from what origin

104
Q

evidence of maturation in monocytic leukemia

A

promonocytes that are blast equivalents

105
Q

blast description of Acute monoblastic and monocytic leukemias

A

1) blast are large w/ abundant, often granular cytoplasm
2) large nuceleoli
3) CD14+, CD4+, CD11b+, CD11c+, CD64

106
Q

clinical presentation of Acute monoblastic and monocytic leukemias

A

1) extramedullary cutaneous, gingival infection
2) bleeding disorder present
3) nonspecific cytogenetic abnormalities are common

107
Q

Acute monoblastic and monocytic leukemias accounts how many percent of AML cases

108
Q

T or F:
Acute monoblastic and monocytic leukemias is common in younger individuals

109
Q

cytochemical staining results of Acute monoblastic and monocytic leukemias

A

1) MPO +
2) SBB +/-
3) NASDA -
4) ANAE +
5) ANBE +

110
Q

FAB classification of pure erythroid leukemia

111
Q

pure erythroid leukemia is aka

A

Di Gugliielmo’s syndrome

112
Q

blast description of pure erythroid leukemia

A

1) MDS with excess blast
2) 80% or more eythroid cell in BM
3) >30% are proerythroblast

113
Q

significant dysplastic features of RBC precursors in M6

A

1) multinucleation
2) megaloblastoid asynchrony
3) vacuolization

114
Q

clinical presentation of pure erythroid leukemia

A

1) complex arrangement of hypodiploid chromosome number are common
2) ring sideroblast,
3) Howell-Jolly body

115
Q

what chromosomes are frequently affected in pure erythroid leukemia

A

chromosome 5 & 7

116
Q

prognosis of pure erythroid leukemia

A

aggressive and rapid

117
Q

cytochemical stain results of pure erythroid leukemia

A

1) MPO +/-
2) SBB +/-
3) NASDA +/-
4) ANAE -
4) ANBE -

118
Q

FAB classification of acute megakaryocytic leukemia

119
Q

requirement for diagnosis of acute megakaryocytic leukemia

A

requires 20% blast, 50% megakaryocyte origin

120
Q

clinical presentation of acute megakaryocytic leukemia

A

1) cytopenia
2) thrombocytosis
3) megakaryoblast size is 3x than small lymphocyte
4) delicate chromatin with prominent nucleoli
5) immature megakaryoblast have light blue cytoplasmic blebs

121
Q

how are megakaryoblasts identified in acute megakaryocytic leukemia

A

immunostaining

122
Q

what are the antibodies are used in immunostaining for acute megakaryocytic leukemia

A

specific for cytoplasmic von Willebrand factor or platelet membrane antigens

123
Q

platelet membrane antigens of acute megakaryocytic leukemia

A

CD41 (glycoprotein IIb)
CD42b (glycoprotein Ib)
CD61 (glycoprotein IIIa)

124
Q

cytochemical stain results of acute megakaryocytic leukemia

A

1) MPO -
2) SBB -
3) NASDA -
4) ANAE -
5) ANBE -

125
Q

identify the AML:
leukemia with no clear evidence differentiation along singe cell line

A

acute leukemia of ambiguous lineage (ALAL)

126
Q

ALAL is commonly referred as

A

acute undifferentiated leukemia (AULs)

127
Q

demonstrate multiplicity of antigens in which it is not possible to determine specific lineage

A

mixed phenotype acute leukemia (MPAL)

128
Q

techniques used to identify AML subtype

A

1) flow cytometry
2) cytogenetic analysis
3) molecular testing

129
Q

old yet important technique used to identify AML subtype

A

cytochemical stain

130
Q

cytochemical stain result of ALL

A

1) MPO +
2) SBB -
3) NASDA -
4) ANBE -/+ (focal)
5) ANAE -‘+ (focal)

130
Q

advantage of cytochemical stains

A

inexpensive

131
Q

T or F:
lymphocyte exhibit MPO activity

132
Q

leukemic myeloblast are usually (positive/negative) for MPO

133
Q

percentage of blast that shows MPO activity

134
Q

auer rods in leukemic blast and promyelocyte test strongly (positive/negative) for MPO

135
Q

maturing granulocyte test strongly (positive/negative) for MPO

136
Q

examples of negative MPO

A

lymphoblast in ALL
lymphoid cells

137
Q

more sensitive cytochemical stain for early myeloid cells

138
Q

granulocytes show (positive/negative result) in SBB

139
Q

why does the SBB stain becomes more intense as the granulocyte mature

A

increase in numbers of primary and secondary granules

140
Q

monocytes show (positive/negative) stain in SBB

A

negative to weakly positive

141
Q

lymphoid cells show (positive/negative) result in SBB

142
Q

how many enzymes of esterases are present in leukocytes

143
Q

esterases are used to differentiate what cells

A

myeloblasts and neutrophilic granulocytes from monocytic origin

144
Q

commonly used substrate esters

A

a-naphtyl acetate and a-naphtyl butyrate

145
Q

T or F:
ANAE and ANBE are specific

A

F
(both are nonspecific)

146
Q

specific esterase reaction used

A

Naphthol AS-D chloroacetate

147
Q

why is NASDA specific

A

only granulocytic cells stain

148
Q

chloroacetate esterase is present in

A

primary granules of neutrophils

149
Q

auer rods show (positive/negative) stain in SBB

150
Q

what does ANAE reveal

A

strong esterase activity in monocytes

151
Q

strong esterase activity demonstrated in ANAE can be inhibited with

A

sodium fluoride

152
Q

granulocyte show (positive/negative) stain in ANAE

153
Q

lymphoid cells show (positive/negative) stain in ANAE

154
Q

monocyte show (positive/negative) stain in ANBE

A

diffuse positive

155
Q

T or F:
ANBE is more sensitive than ANAE

A

F
(less sensitive)

156
Q

granulocytes show (positive/negative) stain in ANBE

157
Q

lymphoid cells show (positive/negative) stain in ANBE

A

negative (although a small positive dot may be seen)

158
Q

T or F:
ANBE is more specific than ANAE

159
Q

positive ANBE indicates what type of leukemia