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Hematology Week 2 > 03.24 - Malignancies from Bone Marrow (Strom) > Flashcards

03.24 - Malignancies from Bone Marrow (Strom) Flashcards

1
Q

14q11 is location of

A

TCR

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2
Q

2 components of CBF heterodimer

A

CBFB and Runx1

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3
Q

3 AML’s dx by genetics alone (regardless of blast count)

A

t(8;21) RUNX1-RUNX1T1; inv(16) CBFB-MYH11; t(15;17) PML-RARA

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4
Q

75% of ALL occur in pts under

A

6

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5
Q

80% of acute leukemias in kids are

A

ALL

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6
Q

Accelerated phase CML will show what in bone marrow

A

Increased blasts

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7
Q

Activating mutation in ET

A

Jak2-v617F or MPL

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8
Q

Activating mutation in jak2 mimics presence of

A

EPO

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9
Q

Activating mutation in P Vera

A

Jak2-V617F in 95% of cases

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10
Q

Auer rods

A

crystallization of granule contents

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11
Q

B-ALL’s lack expression of

A

CD3, CD5

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12
Q

Bad prognosis ALL subgroups

A

t(9;22) BCR-ABL1; t(v;11q23); MLL rearranged

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13
Q

Basic starting point for diff dx

A

PBS manual count

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14
Q

BCR-ABL inhibitor

A

Imatinib

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15
Q

BCR-ABL1 fusion activates

A

proliferation

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16
Q

Bone marrow of CML

A

Hypercellular, many myeloid precursors, little dysplasia, no increase in blasts

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17
Q

Both T- and B-ALL’s express

A

TdT

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18
Q

CBFB-MYH11 is what genetic lesion

A

inv(16)

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19
Q

CD117

A

C-KIT, the SCF receptor

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20
Q

CD19+, CD20+ =

A

Mature lymphocytes/lymphoma

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21
Q

CD34 =

A

Blasts

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22
Q

CD34, CD33 =

A

Myeloid Blasts

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23
Q

Cell increased in Primary Myelofibrosis

A

Platelets

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24
Q

Clinical presentation MLL rearranged ALL

A

Most common leukemia in kids <1

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25
Q

Clinical presentation of BCR-ABL1 ALL

A

Older adults, kids <1

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26
Q

Clinical presentation of CBFB-MYH11 AML

A

Younger patients/kids

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27
Q

Clinical presentation of complex karyotype AML

A

Any age group

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28
Q

Clinical presentation of cyto (-) AML

A

Any age group

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29
Q

Clinical presentation of Essential Thrombocythemia

A

Thrombosis

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30
Q

Clinical presentation of Mastocytosis

A

Variable and Confusing

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31
Q

Clinical presentation of MDS w isolated del(5q)

A

Anemia, often severe; usually elderly, more women

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32
Q

Clinical Presentation of PML-RARA AML

A

Severe thrombocytopenia

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33
Q

Clinical presentation of Polcythemia Vera

A

Thrombosis, HTN, stroke, MI

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34
Q

Clinical Presentation of Primary Myelofibrosis

A

Thrombosis

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35
Q

Clinical presentation of Refractory Anemia w Ring Sideroblasts

A

Unexplained Cytopenia, usually elderly

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36
Q

Clinical presentation of Refractory Anemia with Excess Blasts

A

Cytopenias; usually elderly

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37
Q

Clinical presentation of Refractory Cytopenia w Multilineage Dysplasia

A

Anemia, often severe; usually elderly, more women

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38
Q

Clinical presentation of Refractory Cytopenia w/ Unilineage Dysplasia

A

Unexplained cytopenia(s), usually elderly patients

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39
Q

Clinical presentation of RUNX1-RUNX1T1 AML

A

Younger patients/kids; Pancytopenia symptoms

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40
Q

Clinical presentation of T-ALL

A

25% of pediatric B-ALL; Often w/ thymic mass or lymph node, spleen involvement

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41
Q

Clinical presentation of TEL-AML1 ALL

A

25% of pediatric B-ALL

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42
Q

Common gene in CMML translocations

A

PDGFRB

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43
Q

Common motif for lymphoid malignancies =

A

oncogene&raquo_space;> Ig or TCR promoter

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44
Q

Consequence of IKZF1 mutation

A

Inhibits differentiation

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45
Q

Cytogenetic (-) w/ FLT3 mutation is subtype of what

A

AML

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46
Q

Cytogenetics in MDS w isolated del(5q)

A

Only loss of 5q

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47
Q

Essential thromb can progress to

A

Myelofibrosis, MDS, Acute Leukemia

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48
Q

First criterion for acute leukemia dx

A

Blasts >20%?

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49
Q

Frequency of Jak2 mutation in Essential Thromb

A

50% of cases

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50
Q

Frequency of Jak2 mutation in Primary myelofibrosis

A

50% of cases

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51
Q

Frequency of Jak2 mutation in Primary Myelofibrosis

A

50%

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52
Q

Genetics of Mastocytosis

A

Either cKIT mut or PDGFRA activation (FIP1 trans)

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53
Q

Good prognosis ALL subgroups

A

t(12;21) TEL-AML1/ETV6-RUNX1; Hyperdiploid

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54
Q

How cyto (-) AML is treated depends on

A

results of targeted dna sequencing studies

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55
Q

Hyperdiploid means

A

> 50 chromosomes

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56
Q

Immunophenotye of Essential Thromb

A

No known features

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57
Q

Immunophenotype of BCR-ABL1 ALL

A

CD10+, CD19+, TdT+

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58
Q

Immunophenotype of CBFB-MYH11 AML

A

complicated

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59
Q

Immunophenotype of complex karyotype AML

A

Blast markers (CD34,CD117)+, typically CD33+

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60
Q

Immunophenotype of cyto (-) AML

A

Blast markers (CD34,CD117)+, typically CD33+

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61
Q

Immunophenotype of Mastocytosis

A

Tryptase, CD117 (C-KIT), CD25 usually

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62
Q

Immunophenotype of MLL rearranged MLL

A

CD10-, CD19+, TdT+

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63
Q

Immunophenotype of PML-RARA AML

A

CD13+, CD33+ (all AML +), Weak/absent CD34 (other AML +), HLA-DR-

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64
Q

Immunophenotype of Polcythemia Vera

A

No known features

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65
Q

Immunophenotype of Primary Myelofibrosis

A

No known features

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66
Q

Immunophenotype of Refractory Anemia with excess blasts

A

Blast population (CD34+, and/or CD117+)

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67
Q

Immunophenotype of runx1-runx1t1 AML

A

CD34+, HL-DR+, CD13+, CD33 weak

68
Q

Immunophenotype of T-ALL

A

CD3+, CD5+, TdT+

69
Q

Immunophenotype of TEL-AML1 ALL

A

CD10+, CD20-, CD34+, TdT+

70
Q

In CML, next step after RT-PCR

A

Bone marrow biopsy

71
Q

Intermediate cytogenetic risk group AML is treated based on

A

sequencing

72
Q

inv(16)(p13.1;q22) fuses what genes

A

CBFB-MYH11

73
Q

Lesion in Polcythemia Vera

A

Activating Jak2 mutations in >95% of cases

74
Q

Megakaryocytes in MDS w isolated del(5q)

A

All are mononuclear

75
Q

Mitogen that plays significant role in growth and migration of mast cells

A

SCF

76
Q

More myelocytes than metamyelocytes -

A

Myeloid Bulge (myeloproliferative)

77
Q

Morphology of BCR-ABL1 ALL

A

Big agranular blasts

78
Q

Morphology of CBFB-MYH11 AML

A

Mixed granulocyte-monocyte features; Increased eosinophils in blood and marrow

79
Q

Morphology of complex karyotype AML

A

Undiff, or variably granulocytic, or monocytic/monoblastic

80
Q

Morphology of cyto (-) AML

A

Undifferentiated, or variably granulocytic, or monocytic/monoblastic

81
Q

Morphology of Essential Thromb

A

Increased Megs (large and weird looking, tend to cluster)

82
Q

Morphology of Mastocytosis

A

Aggregates of bland looking cells, round or spindle shaped, sometimes with eosinophilia

83
Q

Morphology of MDS w isolated del(5q)

A

All megs are mononuclear

84
Q

Morphology of MLL rearranged ALL

A

Big agranular blasts

85
Q

Morphology of PML-RARA AML

A

Big blasts; Cleaved “bat wing” nuclei; Many cytoplasmic granules; Auer rods in stacks

86
Q

Morphology of Polcythemia Vera

A

Hypercellular marrow, Erythroid Hyperplasia, Increased Megakaryocytes

87
Q

Morphology of Primary Myelofibrosis

A

Increased megs; Bizarre shapes, Clustering; Fibrosis

88
Q

Morphology of Refractory Anemia with excess blasts

A

Blasts and dyspoietic maturation

89
Q

Morphology of Refractory Anemia with Ring Sideroblasts

A

Ring Sideroblasts, usually w dyspoietic features (in red cell series only)

90
Q

Morphology of Refractory Cytopenia w Multilineage Dysplasia

A

Granulocytes don’t granulate normally; Nuclei don’t lobulate normally

91
Q

Morphology of Refractory Cytopenia w Unilineage Dysplasia

A

Weird precursors, Binucleation or irregular nuclei

92
Q

Morphology of Runx1-Runx1t1 AML

A

Some maturation to myelocytes, Occassional Auer rods

93
Q

Morphology of T-ALL

A

Big agranular blasts

94
Q

Morphology of TEL-AML1 ALL

A

Big agranular blasts

95
Q

Most common leukemia in kids <1

A

MLL rearranged ALL

96
Q

Most common site of systemic involvement in mastocytosis

A

Bone Marrow

97
Q

Most T-ALL’s have translocation of

A

oncogene to a T-cell receptor promoter

98
Q

Myelodysplastic Syndromes usually present as

A

Unexplained cytopenia, bicytopenia, or pancytopenia

99
Q

Myeloid Bulge

A

More myelocytes than metamyelocytes

100
Q

Myeloid bulge leads you to

A

Myeloproliferative

101
Q

Myeloproliferative: Elevated platelets

A

Essential thrombocytopenia, Primary Myelofibrosis

102
Q

Myeloproliferative: Elevated red count =

A

Polycythemia Vera

103
Q

Myeloproliferative: High WBC, All stages of granulocyte maturation in blood =

A

Chronic Myelogenous Leukemia

104
Q

Myeloproliferative: High WBC, Monocytes, Promonocytes, Weird hybrids b/t monocytes and granulocytes =

A

Chronic Myelomonocytic Leukemia (CMML)

105
Q

Myeloproliferative: Increased Eosinophils =

A

Chronic Eosinophilic Leukemia, PDGFR neoplasm

106
Q

Myeloproliferative: Increased mast cells

A

Mastocytosis (primarilty tissue targeted)

107
Q

Myeloproliferative: Increased mature neutrophils

A

Chronic Neutrophilic Leukeia

108
Q

Neoplasms of mast cells usually present as

A

Benign cutaneous lesions in kids (urticaria pigmentosa)

109
Q

Other than Essential Thrombocythemia, elevated platelets can be due to

A

Iron deficiency, infection, chronic inflammation

110
Q

Other than Polcythemia Vera, increased RBC count can be seen in

A

Lung Disease

111
Q

Pathogenesis of CBFB-MYH11 AML

A

Dominant negative repression of myeloid maturation

112
Q

Pathogenesis of MLL rearranged ALL

A

Fusion of transcription regulator to any several partners –> Inhibits differentiation

113
Q

Pathogenesis of Runx1-runx1t1 AML

A

Dominant negative repression of myeloid maturation

114
Q

Patient with myeloid bulge is more likely to have

A

myeloproliferative neoplasm

115
Q

Percent of AML w normal cytogenetics

A

40-50

116
Q

Percent of mastocytoses associated with second heme malig

A

30%

117
Q

PML-RARA is a subtype of what

A

AML

118
Q

PML-RARA is what translocation

A

t(15;17)

119
Q

Polcythemia and Essential thrombocythemia both present with

A

Thrombosis

120
Q

Polycythemia Vera can progress to

A

Myelofibrosis, MDS, Acute Leukemia

121
Q

Poorly functioning clones =

A

Myelodysplastic Syndrome

122
Q

Primary myelofibrosis can progress to

A

Marrow failure, acute leukemia

123
Q

Problem w. immunophenotyping Acute Leukemia

A

Many break the rules, show mixed phenotype

124
Q

Prognosis of BCR-ABL ALL

A

Bad

125
Q

Prognosis of CBFB-MYH11 AML

A

Better than most if “risk adapted therapy is used

126
Q

Prognosis of complex karyotype AML

A

Poor

127
Q

Prognosis of cyto (-) AML

A

Depends on molecular genetics

128
Q

Prognosis of Hyperdiploid ALL

A

Good

129
Q

Prognosis of TEL-AML1 ALL

A

Good

130
Q

Prognsosis of MLL rearranged ALL

A

Bad

131
Q

Progression of MDS w isolated del(5q)

A

10% progress to AML

132
Q

Progression of Refarctory Cytopenia w Mutlilineage Dysplasia

A

10% progress to AML; Median survival 30 months

133
Q

Result of PML-RARA fusion

A

Dominant negative blockade of normal RARA –> inhibits granulocyte differentiation

134
Q

Runx1 is part of a heterodimeric TF called

A

Core Binding Factor (CBF)

135
Q

RUNX1-RUNX1T1 is what translocation

A

t(8;21)

136
Q

SCF

A

Mitogen that plays significant role in growth and migration of mast cells

137
Q

Serum EPO in lung disease vs P Vera

A

elevated in lung disease, not in P Vera

138
Q

Significant morphologic diff between ET and P Vera

A

In ET, megs look “bizarre”

139
Q

Stain helpful for early Primary Myelofibrosis

A

Reticulin –> More reticulin fibers

140
Q

T-ALL’s lack expression of

A

CD20, CD10

141
Q

t(1;19) is what fusion

A

E2A-PBX1

142
Q

t(12;21) is a subtype of what

A

ALL (TEL-AML1)

143
Q

t(12;21) is what fusion

A

TEL-AML1 (ETV6-RUNX1)

144
Q

t(12;21) is what genes

A

TEL-AML1

145
Q

t(15;17) is a subtype of what

A

AML (PML-RARA)

146
Q

t(15;17) is what genes

A

PML-RARA

147
Q

t(16;16) involves what genes

A

CBFB-MYH11 (AML)

148
Q

t(5;14) is what fusion

A

IL3-IgH

149
Q

t(8;21) is a subtype of what

A

AML (RUNX1-RUNXT1)

150
Q

t(8;21) is what genes

A

RUNX1-RUNXT1

151
Q

t(9;22) involves what genes

A

BCR-ABL1

152
Q

t(v;11q23) =

A

MLL rearrangement

153
Q

T/F: Auer Rods can be seen in reactive conditions

A

FALSE

154
Q

T/F: The immunophenotype by itself is a good diagnostic system

A

FALSE

155
Q

Tdt+, CD10 =

A

Lymphoid Blasts

156
Q

Toxic granulation and left shift support what etiology

A

Infectious

157
Q

TP53 mutated AML are typically

A

complex karyotype (unfavorable cytogenetics)

158
Q

Trick for t cell markers

A

single digits

159
Q

Tx of MDS w isolated del(5q)

A

Lenalidomide

160
Q

Tx of PML-RARA fusion

A

ATRA causes differentiation

161
Q

What else has bizarre megs, other than ET

A

Primary Myelofibrosis

162
Q

What is mutated in 84% of BCR-ABL1 ALL

A

IKZF1 TF

163
Q

When mastocytosis spreads beyond skin

A

Release histamine and other mediators –> systemic symptoms (flushing, tachycardia, hypotension)

164
Q

When to suspect myeloproliferative disorders

A

High WBC but no infection

165
Q

Why is knowing features of PML-RARA AML so important

A

Prognosis is good if treated

166
Q

Workup of suspected hematologic disease always begins with

A

review of peripheral smear

Hematology Week 2 (3 decks)

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